NEUROPSYCHOLOGY

Module 01 Basic Concepts of Neuropsychology

Unit 01: Neuropsychological Syndrome

The general failure of psychological tests to provide suitable measures of 'brain damage'
was one of the key factors in moving neuropsychologists in the direction of describing the effects
of cerebral malfunction in terms of syndromes. Piercy (1959) and McFie (1960) favor this
approach-'a patient's performance should be described not so much in terms of extent of deviation
from statistical normality as in terms of extent of approximation to an established syndrome or
abnormality. The use of psychological test methods in the appraisal of the patient's preserved
abilities as well as deficits has helped to clarify the definition of some syndromes and is already
beginning to describe new ones. The syndrome concept has allowed the more realistic use of
psychological test procedures aimed at gauging patterns of impairment on appropriately selected
measures. In discussing the objections to the syndrome method in clinical research as opposed
to practice Kinsbourne (1971) reminds us that the association between the constellation of signs
and symptoms which we term a syndrome and the presence of a disease, is a probabilistic not an
invariant one.
Attentional syndrome
• Hemi Spatial Neglect: Hemi neglect, also known as unilateral neglect, hemi spatial
neglect or spatial neglect is a common and disabling condition following brain damage in
which patients fail to be aware of items to one side of space. Neglect is most prominent
and long-lasting after damage to the right hemisphere of the human brain, particularly
following a stroke. Such individuals with right-sided brain damage often fail to be aware
of objects to their left, demonstrating neglect of leftward items.
• Aprosexia: Represent abnormality to pay attention
• Hyperprosexia: is the abnormal state in which a person concentrates on one thing to the
exclusion of everything else.
• Paraprosexia: is the inability to pay attention to any one thing (a state of constant
distraction).
Memory syndrome
Amnesia: is the loss of memories. These memories may be of events and experiences
that happened in the past few seconds, in the past few days, or even in the distant past.
 You may also be unable to recall experiences after the event that caused your amnesia.
Types

• Retrograde amnesia: Having retrograde amnesia means you've lost your ability to recall
events that happened just before the event that caused your amnesia. Usually this affects
recently made memories, not those from years.

• Anterograde amnesia: If you have this type of amnesia, it means that you can’t
remember new information since your amnesia. You can still recall information from
before the event that caused the amnesia. This is more common than retrograde
amnesia.
• Transient global amnesia (TGA): This is a temporary form of amnesia that tends to
resolve within 24 hours. It occurs more often in middle-aged and older adults. Once
this type of amnesia resolves, it rarely recurs. You may repeatedly ask the same
question and have no recollection of the past few hours. Your memory will slowly
return over the next 24 hours. Experts are unsure what causes this type of amnesia.

• Hyper-amnesia: An extreme degree of retentiveness and recall, with unusual clarity

of memory images.

Movement syndromes

• Apraxia: Apraxia (called "dyspraxia" if mild) is a neurological disorder characterized by

loss of the two ability execute or carry out skilled movements and gestures, despite having
the desire and the physical ability to perform them. Apraxia results from dysfunction of
the cerebral hemispheres of the brain, especially the parietal lobe, and can arise from many
diseases or damage to the brain.
• Ideo-motor Apraxia: Often IMA is a neurological disorder characterized by the inability
to correctly imitate hand gestures and voluntarily mime tool use, (e.g. pretend to brush
one's hair).
• Limb-kinetic apraxia: is the inability to make precise or exact movements with a finger,
an arm or a leg. An example is the inability to use a screwdriver notwithstanding that the
person affected understands what is to be done and has done it in the past.
• Dynamic apraxia: inability to reproduce series of hand movement.
 Visuoperceptual and vasocontractile syndrome
Visual agnosia: Primary visual agnosia is a rare neurological disorder characterized by the
total or partial loss of the ability to recognize and identify familiar objects and/or people by sight.
This occurs without loss of the ability to actually see the object or person. The symptoms of visual
agnosia occur as a result of damage to certain areas of the brain (primary) or in association with
other disorders (secondary).
• Achromatopsia: is a condition characterized by a partial or total absence of color vision.
People with complete achromatopsia cannot perceive any colors; they see only black,
white, and shades of gray. Incomplete achromatopsia is a milder form of the condition
that allows some color discrimination.
• Face blindness, or prosopagnosia: is a brain disorder. It’s characterized by the inability
to recognize or differentiate faces. People with face blindness may struggle to notice
differences in faces of strangers. Others may even have a hard time recognizing familiar
faces.
Language syndrome
• Agraphia: is the loss of the ability to write, loss of ability to produce written language.
• Aphasic agrathia: alteration of the return language deteriorated hand writing, non-
grammatical.
• Pure agrathatic alexia: alteration of the return expression in the absence of other
language disorder.
• Motor agrathsia and visuospatial agrathia: obtaining correct written
communication also depend on motor and visuospatial field. A change in motor or
visuospatial produces quality writing either through the linguistic.
• Alexia: is a rare condition in which reading comprehension is nonexistent or
significantly limited due to brain injury, damage, or trauma.
• Posterior or pure alexia: is an uncommon acquired reading disturbance in which the
loss of the ability to read is not associated with other language deficits.
• Lateral alexia: loss of ability to mean the individual letter that makes up word.
• Central alexia: occurs when reading is affected along with other aspects of language
function (speaking, writing, or comprehension).

• Anterior alexia: Great difficulty naming individual letters of alphabet but can
recognize some written words.
• Aphasia: Aphasia is a problem with speaking, writing, or understanding language. It happens
when you injure parts of the brain that contribute to language. The language areas of the brain
include the frontal lobe, the temporal lobe, and the parietal lobe.

• Broca's Aphasia/Motor Aphasia: Broca's aphasia makes it difficult for a person to

express themselves, but they usually are able to understand language. Because strokes
that cause Broca's aphasia often damage other areas of the brain, people may also have
difficulties with movement, reading, and writing.

• Wernicke’s Aphasia: Wernicke’s aphasia is named after the person who discovered
the areas of the brain that are responsible for our ability to understand language. These
areas are located in the temporal lobe.
People with Wernicke’s aphasia can’t understand others, or even themselves, when
they speak.
• Global Aphasia: This is a type of aphasia that occurs when damage in the brain is so
widespread that it involves both Broca's and Wernicke’s language areas. Survivors
with global aphasia are unable to understand spoken language or to speak at all. In
some cases, people with global aphasias can still communicate by using written
language.
• Transcortical sensory aphasia (TSA): is a kind of aphasia that involves damage to
specific areas of the temporal lobe of the brain, resulting in symptoms such as poor
auditory comprehension, relatively intact repetition, and fluent speech with semantic
paraphasias present.
• Transcortical Motor Aphasia: is a type of non-fluent aphasia. This means that speech
is halting with a lot of starts and stops. People with TMA typically have good repetition
skills, especially compared to spontaneous speech.
Functional systems

The idea of a functional system as the neurological underpinning of a complex

psychological function has been developed over a long period by Luria and is clearly outlined
in his recent textbook (Luria, 1973b). At the outset he draws attention to the fact that the term
'function' may be used in at least two principal ways. Firstly one may describe the function of
particular cells or organs, e.g.one of the functions of the liver is to produce bile, the function
of the islet cells of Langerhans is to produce insulin. This usage is readily understood. On the
other hand, the term function is widely used to describe more complex processes involving the
integrated participation of a number of tissues and organs in a functional process, e.g., the
function of digestion, circulation, and respiration.
 Such organizations are termed systems and though the final result such as the absorption of
nourishment, or the provision of oxygen to the tissues remains constant, the way in which the
system performs the function varies considerably according to a wide variety of factors. The
presence of a constant (invariant) task, performed by variable (variative) mechanisms, bringing
the process to a constant (invariant) result, is one of the basic features distinguishing the work of
every functional system.

The systemic approach has a second advantage which proves useful in topical diagnosis. While
it is true that damage in any part of a functional system may lead to disruption of a psychological
process it is also true that damage to different parts of a system will impress a different character
on the complex of symptoms and signs which result from the damage. Thus it is of paramount
importance to establish not only that there is an alteration in a particular psychological function
following a brain lesion but also what qualitative features this loss of function has. It was
principally for this reason that psychological tests, particularly some of the more widely used
psychometric measures proved of such little value in diagnosis since they did not allow this
difference in the quality of performance to be brought out or psychologists were too impressed
with the scores or level of performance to see the significance of qualitative changes. Indeed
workers like Goldstein were roundly attacked because of their lack of norms, standardization
and other features of the because of their lack of norms, standardization and other features of
the epitome of psychological assessment, the intelligence test. This over generalization is easy
to make with hindsight and it is true, particularly of the British clinical psychologists that they
quite early realized the shortcomings of dependence upon test scores alone. Shapiro (1951)
expressed it pithily when he commented 'the test scores do not communicate the responses in
full."

The notion of functional systems is a marked advance on the notion of strict localization of
function in discrete areas of the cortex. The functional system has as its anatomical basis a
number of cortical and subcortical areas working' in concert through the action of fiber pathways
and it is for this reason that a working knowledge of the gross anatomy of the brain will be
indispensable for the neuropsychologist.
 Unit 2 Disconnection syndrome and double dissociation of function

The term “disconnection syndrome” is applied to the effects of lesions of

association pathways, either those which lie within a single cerebral hemisphere or those
which join the two halves of the brain (Geschwind,1965). Often the disconnection
syndromes are talked about more generally as Callosal Syndromes. The corpus callosum is
by far the largest of the nerve fiber bundles that directly connect one cerebral hemisphere
with the other. These are generally called cerebral commissures and include the anterior and
hippocampal commissures. These syndromes occur when the corpus callosum is partially or
fully surgically divided. Syndromes of hemisphere disconnection can also occur when there
is a partial disconnection such as in naturally occurring diseases/condition such as stroke
(thrombosis etc.) Callosal lesions are often accompanied by damage to neighboring
structures. Therefore “neighborhood signs” may overshadow signs of callosal
disconnection.
ROGER SPERRY SPLIT BRAIN EXPERIMENTS (1959–1968)
In the 1950s and 1960s, Roger Sperry performed experiments on cats, monkeys, and
humans to study functional differences between the two hemispheres of the brain in the United
States. To do so he studied the corpus callosum, which is a large bundle of neurons that connects
the two hemispheres of the brain. Sperry severed the corpus callosum in cats and monkeys to
study the function of each side of the brain. He found that if hemispheres were not connected,
they functioned independently of one another, which he called a split-brain. The split-brain
enabled animals to memorize double the information. Later, Sperry tested the same idea in
humans with their corpus callosum severed as treatment for epilepsy, a seizure disorder. He
found that the hemispheres in human brains had different functions. The left hemisphere
interpreted language but not the right. Sperry shared the Nobel Prize in Physiology or Medicine
in 1981for his split-brain research.

Sperry also studied other aspects of brain function and connections in mammals and humans,
beyond split-brains, in 1940s and 1950s. In 1963, he developed the chemoaffinity hypothesis,
which held that the axons, the long fiber-like process of brain cells, connected to their target
organs with special chemical markers. This explained how complex nervous systems could
develop from a set of individual nerves. Sperry then also studied brain patterns in frogs, cats,
monkeys, and human volunteers.
 Sperry began his research on split-brain in late 1950s to determine the function of the corpus
callosum. He noted that humans with a severed corpus callosum did not show any significant
difference in function from humans with intact corpus callosum, even though their hemispheres
could not communicate due to the severing of the corpus callosum. Sperry postulated that there
should be major consequences from cutting the brain structure, as the corpus callosum
connected the two hemispheres of the brain, was large, and must have an important function.
Sperry began designing experiments to document the effects of a severed corpus callosum. At
the time, he knew that each hemisphere of the brain is responsible for movement and vision on
the opposite side of the body, so the right hemisphere was responsible for the left eye and vice
versa. Therefore, Sperry designed experiments in which he could carefully monitor what each
eye saw and therefore what information is was going to each hemisphere.
Sperry experimented with cats, monkeys, and humans. His experiments started with split-
brain cats. He closed one of their eyes and presented them with two different blocks, one of
which had food under it. After that, he switched the eye patch to the other eye of the cat and put
the food under the other block. The cat memorized those events separately and could not
distinguish between the blocks with both eyes open. Next, Sperry performed a similar
experiment in monkeys, but made them use both eyes at the same time, which was possible due
to special projectors and light filters. The splitbrain monkeys memorized two mutually exclusive
scenarios in the same time a normal monkey memorized one. Sperry concluded that with a
severed corpus callosum, the hemispheres cannot communicate and each one acts as the only
brain.

Sperry moved on to human volunteers who had a severed corpus callosum. He showed a word
to one of the eyes and found that split-brain people could only remember the word they saw with
their right eye. Next, Sperry showed the participants two different objects, one to their left eye
only and one to their right eye only and then asked them to draw what they saw. All participants
drew what they saw with their left eye and described what they saw with their right eye. Sperry
concluded that the left hemisphere of the brain could recognize and analyze speech, while the
right hemisphere could not.

In the 1960s when Sperry conducted his split-brain research on humans, multiple
scientists were studying brain lateralization, the idea that one hemisphere of the brain is better at
performing some functions than the other hemisphere. However, researchers did not know which
tasks each side of the brain was responsible for, or if each hemisphere acted independently from
the other.

Sperry describes his research in cats in the article "Cerebral Organization and Behavior"
published in 1961. To test how the cutting of the corpus callosum affected mammals, Sperry cut
the corpus callosum of multiple cats and had them perform some tasks that involved their vision
and response to a visual stimulus. After severing each cat´s corpus callosum, he covered one of
the cat´s eyes to monitor with which eye the cat could see. Sperry could switch the eye patch from
one eye to the other, depending on which visual field he wanted the cat to use. Next, Sperry
showed the cats two wooden blocks with different designs, a cross and a circle. Sperry put food
for the cat under one of the blocks. He taught the cats that when they saw the blocks with one eye,
for instance, the right eye, the food was under the circle block, but when they saw it with the left
eye, the food was under the block with a cross. Sperry taught the cats to differentiate between
those two objects with their paws, pushing the correct wooden block away to get the food.
When Sperry removed the eye patch and the cats could see with both eyes, he performed the same
experiment. When the cats could use both eyes, they hesitated and then chose both blocks almost
equally. The right eye connects to the left hemisphere and the left eye connects to the right
hemispheres. Sperry suspected that since he cut the corpus callosum in those cats, the hemispheres
could not communicate. If the hemispheres could not communicate and the information from one
eye only went to one hemisphere, then only that hemisphere would remember which block usually
had food under it. From that, Sperry concluded that the cats remembered two different scenarios
with two different hemispheres. He suspected that the cats technically had two different brains,
as their hemispheres could not interact and acted as if the other one did not exist.

Sperry performed a similar experiment with monkeys, in which he also cut their corpus
callosum. He wanted to test if both hemispheres could operate at the same time, even though
they were not connected. That required separation of visual fields, or making sure that the right
eye saw a circle, while the left eye saw a cross, like in the cat experiment, but without an eye
patch and both eyes would see something at the same time instead of interchanging between the
open eyes. Sperry solved that by using two projectors that were positioned side-by-side at an
angle and showed mutually exclusive images. For example, the projector on the right showed a
circle on the left and a cross on the right, while the projector on the left showed a cross on the
left and a circle on the right. Sperry placed special light filters in front of each of the monkey´s
eyes. The light filters made it so that each eye saw the images from only one of the projectors.
That meant one of the eyes saw the circle on the right and the cross on the left, while the other
eye saw the cross on the right and the circle on the left. From his experiments with cats, Sperry
knew that there was no sharing of information from right and the left hemispheres, so he made
the monkeys memorize two different scenarios at the same time.

The left eye saw a scenario where food would be dispersed when the monkey pressed the
button corresponding to a cross, while the right eye saw a scenario where food would be
dispersed when the monkey pressed a button corresponding to a circle. Ultimately, it was the
same button, but the eyes saw it differently because of two projectors and special light filters.
Sperry concluded that both hemispheres of the brain were learning two different, reversed,
problems at the same time. He noted that the split-brain monkeys learned two problems in the
time that it would take a normal monkey to learn one, which supported the assumption that the
hemispheres were not communicating and each one was acting as the only brain. That seemed as
a benefit of cutting corpus callosum, and Sperry questioned whether there were drawbacks to the
procedure.

Sperry performed the next set of experiments on human volunteers, who had their corpus callosum
severed previously due to outside factors, such as epilepsy. Sperry asked volunteers to perform
multiple tests. From his previous experiments with cats and monkeys, Sperry knew that one, the
opposite, hemisphere of the brain would only analyze information from one eye and the
hemispheres would not be able to communicate to each other what they saw. He asked the
participants to look at a white screen with a black dot in the middle. The black dot was the dividing
point for the fields of view for a person, so the right hemisphere of the brain analyzed everything
to the left of the dot and the left hemisphere of the brain analyzed everything that appeared to the
right of the dot. Next, Sperry showed the participants a word on one side of the black dot for less
than a second and asked them to tell him what they saw. When the participants saw the word with
their right eye, the left hemisphere of the brain analyzed it and they were able to say what they
saw. However, if the participants saw the word with their left eye, processed by right hemisphere,
they could not remember what the word was. Sperry concluded that the left hemisphere could
recognize and articulate language, while the right one could not.

Sperry then tested the function of the right hemisphere. He asked the participants of the same
experiment that could not remember the word because it was in the left visual field to close their
eyes and draw the object with their left hand, operated by the right hemisphere, to which he
presented the word. Most people could draw the picture of the word they saw and recognize it.
Sperry also noted that if he showed the word to the same visual field twice, then the person would
recognize it as a word they saw, but if he showed it to the different visual fields, then the
participants would not know that they saw the word before. Sperry concluded that the left
hemisphere was responsible not only for articulating language, but also for understanding and
remembering it, while the right hemisphere could only recognize words, but was not able to
articulate them. That supported the previously known idea that the language center was in the left
hemisphere.

Sperry performed another similar experiment in humans to further study the ability of the
right hemisphere to recognize words. During that experiment, Sperry asked volunteers to place
their left hand into a box with different tools that they could not see. After that, the participants
saw a word that described one of the objects in the box in their left field of view only. Sperry
noted that most participants then picked up the needed object from the box without seeing it, but
if Sperry asked them for the name of the object, they could not say it and they did not know why
they were holding that object. That led Sperry to conclude that the right hemisphere had some
language recognition ability, but no speech articulation, which meant that the right hemisphere
could recognize or read a word, but it could not pronounce that word, so the person would not
be able to say it or know what it was.
In his last series of experiments in humans, Sperry showed one object to the right eye of
the participants and another object to their left eye. Sperry asked the volunteers to draw what
they saw with their left hand only, with closed eyes. All the participants drew the object that they
saw with their left eye, controlled by the right hemisphere, and described the object that they saw
with their right eye, controlled by the left hemisphere. That supported Sperry´s hypothesis that
the hemispheres of brain functioned separately as two different brains and did not acknowledge
the existence of the other hemisphere, as the description of the object did not match the drawing.
Sperry concluded that even though there were no apparent signs of disability in people with a
severed corpus callosum, the hemispheres did not communicate, so it compromised the full
function of the brain.

Sperry received the 1981 Nobel Prize in Physiology or Medicine for his split-brain
research. Sperry discovered that the left hemisphere of the brain was responsible for language
understanding and articulation, while the right hemisphere could recognize a word, but could not
articulate it. Many researchers repeated Sperry´s experiments to study the split-brain patterns and
lateralization of function.
DISCONNECTING COGNITIVE FUNCTIONS
1. The anatomically defined cortical lobes each engage in wide range of cognitive activities-
for example, temporal lobe appears to play a significant role not only in vision and audition
but also in more complex cognitive functions such as memory, language and emotion
• 2. Although the cortical lobes engage in different cognitive activities, they overlap
remarkably in function, for example, the multimodal frontal lobe cells responds to visual
auditory, somatosensory, olfactory and taste inputs and participate in such functions as
memory language and emotion.
• 3.The cutting of cerebral connections is called disconnection, and the ensuing behavioural
effects are called disconnection syndromes. The behavioural changes that result from
disconnecting cerebral regions can be odd, and they differ from behavioural changes we
might expect if either area were damaged but remained connected.
ANATOMY OF CEREBRAL CONNECTIONS
There are three major types of neural fiber pathways that connect the neocortex:

1. Association Pathways- distinguished as either a) long fiber bundles that connect distant
neocortical areas or b) short, subcortical, U shaped fibers that connect adjacent neocortical
areas

2. Projection Pathways- includes ascending fibers from lower brain centers to the neocortex,
such as projection from thalamus, and descending fibers from the neocortex to the
brainstem and spinal cord.

3. Commissural Pathways- connect the two hemispheres and include principally the corpus
callosum, anterior commissure and hippocampal commissures. The corpus callosum (from
the Latin callus, meaning “hard body”) provides the major connection of neocortical areas.
In humans, it is made up of 200 million to 800 million fibers. About half are unmyelinated
and quite small. Most, but not all, areas of the two hemispheres are connected.

BEHAVIOURAL EFFECTS OF DISCONNECTION

• The clinical effects of callosal disconnection were first seriously considered by Carl
Wernicke in 1874 and became a prominent part of early neurology.
• Wernicke predicted the existence of an aphasic syndrome, conduction aphasia, that would
result from severing fiber connections between the anterior and posterior speech zones.
• In this condition, speech sounds and movements are retained, but speech is impaired
because it cannot be conducted from one region to the other.
In 1892, Joseph Dejerine was the first to demonstrate a distinctive behavioural deficit
resulting from pathology of the corpus callosum.
• In a series of paper published around 1900, Hugo Liepmann most clearly demonstrated
the importance of severed connections as an underlying factor in the effects of cerebral
damage.
• Having carefully analysed the behaviour of a particular patient, Liepmann predicted a
series of neocortical disconnections that could account for the behaviour.
• In 1906, after the patient dies, Liepmann published the post-mortem findings, which
supported his hypothesis.
• Liepmann wrote extensively on the principle of disconnection, particularly about the idea
that some apraxias might result from disconnection.
• He reasoned that if a patient was given a verbal command to use the left hand in a particular
way, only the verbal left hemisphere would understand the command.
• To move the left hand, a signal would then have to travel from the left hemisphere through
the corpus callosum to the right hemispheric region that controls movements of the left
hand, interrupting the part of the corpus callosum that carries the command from the left
hemisphere to the right would disconnect the right hemisphere’s motor region from the
command.
• Thus, although the subject would comprehend the command, the left hand would be unable
to obey it. The apraxia would occur in the absence of the weakness or incoordination of
the left hand that would develop in the presence of right hemisphere motor cortex, which
controls the actual movement of the left hand.
• In the early 1950s, an important series of papers by Ronald Myers and by Roger Sperry
(e.g., Myers, 1956: Glickstein and Sperry, 1960) revived interest in the effects of
disconnecting neocortical regions. They examined the behavioural effects of severing the
corpus callosum of the cat.
• Myers and Sperry’s results revealed that under special training procedures, the animals
could be shown to have severe deficits.
• If sensory information was allowed separate access to each hemisphere, each hemisphere
could be shown to have its own independent perceptual, learning and memory processes.
• The corpus callosum does indeed serve an important function, a conclusion confirmed in
subsequent studies by Sperry & his colleagues on the effects of surgical disconnection of
the cerebral hemispheres of humans for the treatment of intractable epilepsy.
• The success of the Myers and Sperry experiments stimulated interest in other connections
within the brain.
• Geschwind (1965) began to reassess the clinical effects of naturally occurring neocortical
lesions as possibly indicating disconnections of various regions of cerebral hemispheres
(see review by Catoni and ffytche, 2005).
• In parallel work, Mishkin (1979) began to construct animal models of human
disconnection syndromes by disconnecting related neocortical regions from one another.
These researchers have demonstrated the critical interdependence of these normally
connected regions.
Disconnection Syndromes based on the work of NORMAN GESCHWIND

1. Word blindness without agraphia: Regarded as the first example of a callosal

disconnection syndromes for which clear anatomical evidence was established.

2. Pure Word Deafness: A lesion, located subcortically in the left temporal lesion typically
destroys the left auditory radiation as well as callosal fibers from the opposite auditory
region. The lesion has the effect of preventing the speech area (that part of the auditory
association cortex that is Wernicke’s area) from receiving auditory stimulation. Although
the right primary auditory cortex could receive auditory stimuli it can’t convey them to the
speech area because the callosal connections from the right side were destroyed in the left
temporal lobe.

3. Lesions of Wernicke’s Area: although verbal comprehension is impaired, speech is also

impaired. The loss of Wernicke’s area can be regarded as the destruction of a memory
store as it was in fact regarded classically. Presumably it functions importantly as the
“storehouse” of auditory associations. Geschwind describes that “a name” passes through
Wernicke’s area then via the angular gyrus which arouses associations and other parts of
the brain. Wernicke’s area attains its essential importance and comprehension as it is also
involved in the arousal of associations. (maybe we should discuss this and talk about
whether this is phenomena is discussed in this way anymore).
4. Tactile aphasia: A disturbance characterized by an inability to name objects tactilely in
the preservation of the ability to name on the basis of visual or auditory stimulation and
spontaneous speech.
Double dissociation function
In neuropsychology, dissociation involves identifying the neural substrate of a
particular brain function through identification of case studies, neuroimaging, or
neuropsychological testing.
One method is "double dissociation". In a series of patients with traumatic brain
injury, one might find two patients, A and B. Patient A has difficulty performing
cognitive tests for, say auditory memory but has no problem with visual memory. Patient
B has the opposite problem. By using neuroimaging (or neuropathology post mortem) to
identify the overlap and dissociation between lesioned areas of the brain, one can infer
something about the localization of function in the normal brain.
Broca and Wernicke were two physicians of the 1800s. In observation of their
patients an apparent double dissociation was observed. Broca's patients could no longer
speak but could understand language (non-fluent aphasia) while Wernicke's patients
could no longer understand language but could produce jumbled speech (fluent aphasia).
Postmortems revealed lesions in separate areas of the brain in each case (now referred to
as Broca's and Wernicke's areas respectively). Although the neurophysiology of language
is now known to be more complicated than described by Broca or Wernicke, this classic
double dissociation acted to begin modern neuropsychological investigation of language.
Dr. Oliver Sacks has described many famous cases in his books. There are patients
who cannot name an object when they can only see it, but can when they use other senses
like touching or smelling. Patient D.F. was unable to place a card in a slot, but could do
so when told to place it "as if mailing a letter". From this the conclusion was drawn that
judging orientation is one ability (which D.F. had lost) and visual control of an action
another (which D.F. could still do).

To make the difference between single and double dissociations easier to understand,
Parkin [2] gives the following example:
If your TV set suddenly loses the color you can conclude that picture transmission and
color information must be separate processes (single dissociation: they cannot be
independent because you cannot lose the picture and still have the color). If on the other
 hand you have two TV sets, one without sound and one without a picture you can conclude
that these must be two independent functions (double dissociation).

The term, first proposed by Hans-Lukas Teuber (1955), refers to a situation in which a
lesion of brain area A impairs function 1 but not function 2, while a lesion of brain area B
produces the reverse pattern.

A classic example of Double Dissociation is speech and language comprehension.

Although both processes pertain to use of language, the brain structures that control them
work independently. When a part of the brain called the Broca's area is damaged, patients
may still understand language but be unable to speak fluently. They know what they want
to say, but are unable to express themselves. On the other hand, when a part of the brain
called Wernicke's area is damaged, patients may still speak fluently, but be unable to
comprehend language. This results in properly constructed but nonsensical sentences.

Unit 3: Brain plasticity, cerebral dominance, lateralization of function.

BRAIN PLASTICITY

• Plasticity or neuroplasticity is the lifelong ability of the brain to reorganize the neural
pathways i.e., changing of the structure, function, and organization of neurons in response
to the new experiences.
• It refers to strengthening or weakening nerve connections or adding new nerve cells based
on new experiences.
• In the nineteenth century, the work of French physiologist Pierre Flourens (1794–1867)
and, later, the German physiologist Friedrich L. Goltz (1834– 1902) once again challenged
the idea that brain functions are localized (Flou- rens, 1960; Goltz, 1960). Both men
created animal models of human clinical cases by removing small regions of cortex. Both
expected that the animals would lose specific functions. Flourens found instead that with
the passage of time, his animals recovered from their initial impairments to the point that
they seemed to behave typically. Thus, an impaired pigeon that would not initially eat or
fly with time recovered both abilities. More dramatically, Goltz removed almost the entire
cortex and a good deal of underlying brain tissue from three dogs that he studied for 57
days, 92 days, and 18 months, respectively, until each dog died. The dog that survived for
18 months was more active than a typical dog. Its periods of sleep and waking were shorter
than normal, but it still panted when warm and shivered when cold. It walked well on
 uneven ground and was able to regain its balance when it slipped. If placed in an abnormal
posture, it corrected its position. After hurting a hind limb on one occasion, this dog trotted
on three legs, holding up the injured limb. It was able to orient to touches or pinches on its
body and to snap at the object that touched it, although its orientations were not very
accurate. If offered meat soaked in milk or meat soaked in bitter quinine, it accepted the
first and rejected the second. It responded to light and sounds, although its response
thresholds were elevated; that is, its senses were not as acute as those typical of a dog.
Although impaired, its recovered abilities clearly suggested that the remaining brainstem
could substitute for the cortex. These early experiments actually built the foundation for
neuropsychology’s emphases on recovery of function and on promoting recovery by
rehabilitation after brain damage, even in extreme circumstances, as illustrated in the
Snapshot. Neuropsychologists recognize that, although all function may not be recovered
after injury, the brain’s plasticity can be harnessed to produce significant functional
improvements.
• The human brain is composed of approximately 86 billion neurons. Early researchers
believed that neurogenesis, or the creation of new neurons, stopped shortly after birth.
Today, it's understood that the brain possesses the remarkable capacity to reorganize
pathways, create new connections, and, in some cases, even create new neurons—a
concept called neuroplasticity, or brain plasticity.

There are two main types of neuroplasticity.

Functional plasticity: The brain's ability to move functions from a damaged area of the
brain to other undamaged areas

Structural plasticity: The brain's ability to actually change its physical structure as a
result of learning

There are many benefits of brain neuroplasticity. It allows your brain to adapt and change,
which helps promote: The ability to learn new things, The ability to enhance your existing
cognitive capabilities, Recovery from strokes and traumatic brain injuries, Strengthening
areas if some functions are lost or decline, Improvements that can promote brain fitness

•In some cases, patients with brain damage have healed naturally because healthy nerves
took on the task of damaged or destroyed nerves, allowing for at least some level of
functionality.
•There are variety of mechanisms by which neural plasticity can occur

Axonal sprouting is a process where fine nerve processes – sprouts – grow out from the
intact axons to reinnervate denervated muscle fibers. Thereby the sprouting sustains the
nerve supply to muscles and, in turn, the ability to move.

• synaptic pruning. synaptic pruning, refers to the process by which extra neurons and
synaptic connections are eliminated in order to increase the efficiency of neuronal
transmissions. some connections are strengthened while others are eliminated. This
process is known as synaptic pruning.

•While plasticity occurs throughout the lifetime, certain types of changes are more
predominant at specific ages. The brain tends to change a great deal during the early years
of life, for example, as the immature brain grows and organizes itself.

•Generally, young brains tend to be more sensitive and responsive to experiences than
much older brains. But this does not mean that adult brains are not capable of adaptation.

•Genetics can also have an influence. The interaction between the environment and
genetics also plays a role in shaping the brain's plasticity.

•Plasticity is ongoing throughout life and involves brain cells other than neurons, including
glial and vascular cells. It can occur as a result of learning, experience, and memory
formation, or as a result of damage to the brain.

•While people used to believe that the brain became fixed after a certain age, newer
research has revealed that the brain never stops changing in response to learning.

•In instances of damage to the brain, such as during a stroke, the areas of the brain
associated with certain functions may be damaged. Eventually, healthy parts of the brain
may take over those functions and the abilities can be restored.

Brain Plasticity Also Has Limitations

It is important to note, however, that the brain is not infinitely malleable. Certain areas of
the brain are largely responsible for certain actions. For example, there are areas of the
brain that play critical roles in things such as movement, language, speech, and cognition.
Damage to key areas of the brain can result in deficits in those areas because, while some
recovery may be possible, other areas of the brain simply cannot fully take over those
functions that were affected by the damage.
Early Theories
Up until the 1960s, researchers believed that changes in the brain could only take place
during infancy and childhood. By early adulthood, it was believed that the brain's physical
structure was mostly permanent. In his 2007 book, "The Brain that Changes Itself: Stories
of Personal Triumph From the Frontiers of Brain Science," which took a historical look at
early theories, psychiatrist and psychoanalyst Norman Doidge suggested that this belief
that the brain was incapable of change primarily stemmed from three major sources,
including:
An ancient belief that the brain was much like an extraordinary machine, capable of
astonishing things yet incapable of growth and change .The inability to actually observe
the microscopic activities of the brain The observation that people who had suffered
serious brain damage were often unable to recover.

Early on, the psychologist William James had suggested that the brain was perhaps not as
unchanging as previously believed. Way back in 1890, in his book "The Principles of
Psychology," he wrote, "Organic matter, especially nervous tissue, seems endowed with
a very extraordinary degree of plasticity." However, this idea went largely ignored for
many years.

Modern Theories
In the 1920s, researcher Karl Lashley found evidence of changes in neural pathways of
rhesus monkeys. By the 1960s, researchers began to explore cases in which older adults
who had suffered massive strokes were able to regain functioning, demonstrating that the
brain was more malleable than previously believed. Modern researchers have also found
evidence that the brain is able to rewire itself following damage.
Modern research has demonstrated that the brain continues to create new neural pathways
and alter existing ones in order to adapt to new experiences, learn new information, and
create new memories.
Thanks to modern advances in technology, researchers are able to get a never-before-
possible look at the brain's inner workings. As the study of modern neuroscience
flourished, a body of research has demonstrated that people are not limited to the mental
 abilities they are born with and that damaged brains are often quite capable of remarkable
change.
There are things you can do to help encourage your brain to adapt and change. Some of
the ways that you can utilize neuroplasticity in beneficial ways include:
Enriching Your Environment
Learning environments that offer plenty of opportunities for focused attention, novelty,
and challenge have been shown to stimulate positive changes in the brain. This is
particularly important during childhood and adolescence, but enriching your environment
can continue to provide brain rewards well into adulthood.1
Things you can try include:
Learning a new language, Learning how to play an instrument, Traveling and exploring
new places, Creating art and other creative pursuits, Reading, Getting Plenty of Rest
Research has shown that sleep plays an important role in dendritic growth in the brain.2
Dendrites are the growths at the end of neurons that help transmit information from one
neuron to the next. By strengthening these connections, you may be able to encourage
greater brain plasticity.Sleep has been shown to have important effects on both physical
and mental health. You can find ways to improve your sleep by practicing good sleep
hygiene.
Exercise

Regular physical activity has been shown to have a number of brain benefits.
Some research suggests that exercise might help prevent neuron losses in key areas of the
hippocampus, a part of the brain involved in memory and other functions. One 2018
review published in the journal Frontiers in Neuroscience suggested that exercise could
also play a role in neurogenesis in the hippocampal region.

LATERALIZATION OF FUNCTION

A now legendary chain of observations and speculations led to confirmation that language
is both localized in the brain and lateralized, that is, located on one side of the brain. This
discovery led to the principle of lateralization of function, that one cerebral hemisphere
can perform a function not shared by the other (Benton, 1964). We begin on February 21,
1825, as French physician Jean Baptiste Bouillaud (1796–1881) read a paper before the
Royal Academy of Medicine in France. Bouillaud argued from clinical studies that certain
functions are localized in the cortex and, specifically, that speech is localized in the frontal
lobes, in accordance with Gall’s theory. Observing that acts such as writing, drawing,
painting, and fencing are car- ried out with the right hand, Bouillaud also suggested that
the part of the brain that controls them might be the left hemisphere. Physicians had long
recognized that damage to a hemisphere of the brain impairs movement of the opposite
side of the body. A few years later, in 1836, Marc Dax (1770–1837) presented a paper in
Montpellier, France, discussing a series of clinical cases demonstrat- ing that disorders of
speech were constantly associated with lesions of the left hemisphere. Dax’s manuscript
received little attention, however, and was not published until 1865, by his son. Ernest
Auburtin (1825–1893), Bouillaud’s son-in-law, supported Bouillaud’s cause. At a meeting
of the Anthropological Society of Paris in 1861, he reported the case of a patient who lost
the ability to speak when pressure was applied to his exposed frontal lobe. Auburtin also
described another patient, ending with a promise that other scientists interpreted as a
challenge: For a long time during my service with M. Bouillaud I studied a pa- tient,
named Bache, who had lost his speech but understood everything said to him and replied
with signs in a very intelligent manner to all questions put to him. . . . I saw him again
recently and his disease has progressed; slight paralysis has appeared but his intelligence
is still un- impaired, and speech is wholly abolished. Without a doubt this man will soon
die. Based on the symptoms that he presents we have diagnosed softening of the anterior
lobes. If, at autopsy, these lobes are found to be intact, I shall renounce the ideas that I
have just expounded to you. (Stookey,
1954) Paul Broca, founder of the Society, heard Auburtin’s challenge. Five days later he
received a patient, a Monsieur Leborgne, who had lost his speech and was able to say only
“tan” and utter an oath. The right side of his body was par- alyzed, but he seemed
intelligent and typical in other respects. Broca recalled Auburtin’s challenge and invited
him to examine Tan, as the patient came to be called. Together they agreed that, if
Auburtin was right, Tan should have a frontal lesion. Tan died on April 17, 1861, and the
next day Broca (1960) submitted his findings to the Anthropological Society. (This
submission is claimed to be the fastest publication ever made in science.) Auburtin was
correct: the left frontal lobe was the focus of Tan’s lesion. By 1863, Broca had col- lected
eight more cases similar to Tan’s, all with a frontal lobe lesion in the left hemisphere
(Broca, 1865). As a result of his studies, Broca located speech in the third convolution
(gyrus) of the frontal lobe on the left side of the brain (Figure 1.6A). By demonstrating
that speech is located only in one hemisphere, Broca discovered the brain property of
 functional lateralization. Because speech is thought central to human consciousness, the
left hemi- sphere is frequently referred to as the dominant hemisphere to recognize its
special role in language (Joynt, 1964). In recognition of Broca’s contribution, the anterior
speech region of the brain is called Broca’s area, and the syndrome that results from its
damage is called Broca’s aphasia (from the Greek a, for “not,” and phasia, for “speech”).
An interesting footnote: Broca examined Tan’s brain (Figure 1.6B) only by inspecting its
surface. His anatomical analysis was criticized by French anatomist Pierre Marie (1906),
who reexamined the preserved brains of Br- oca’s first two patients, Tan and
Monsieur Lelong, 25 years after Broca’s death. Marie pointed out in his article, “The Third
Left Frontal Convolution Plays No Particular Role in the Function of Language,” that
Lelong’s brain showed general nonspecific atrophy, common in senility, and that Tan had
additional extensive damage in his posterior cortex that may have accounted for his
aphasia. Broca was aware of Tan’s posterior damage but concluded that, whereas it
contributed to his death, the anterior damage had occurred earlier, producing his aphasia.
Broca’s view on localization and his discovery of lateralization be- came dogma in
neuropsychology for the next 100 years, but a dogma tempered by Pierre Marie’s
criticism.

A Lateralized Language Model

German anatomist Carl Wernicke (1848–1904) created the first model of how the brain
produces language in 1874. Wernicke was aware that the part of the cortex into which the
sensory pathway from the ear projects— the auditory cortex—is located in the temporal
lobe behind Broca’s area. He therefore sus- pected a relation between hearing and speech
functioning, and he described cases in which aphasic patients had lesions in this auditory
area of the temporal lobe. These patients displayed no opposite-side paralysis. (Broca’s
aphasia is fre- quently associated with paralysis of the right arm and leg, as described for
Tan.) They could speak fluently, but what they said was confused and made little sense.
(Broca’s patients could not articulate, but they seemed to understand the meaning of
words.) Although Wernicke’s patients could hear, they could neither understand nor repeat
what was said to them. Wernicke’s syndrome is sometimes called temporal-lobe aphasia
or fluent aphasia, to emphasize that the person can say words, but is more frequently called
Wernicke’s aphasia. The associated region of the temporal lobe is called Wernicke’s area.
Wernicke’s model of language organization in the left hemisphere is illus- trated in Figure
 1.7A. He proposed that auditory information travels to the temporal lobes from the
auditory receptors in the ears. In Wernicke’s area, sounds are processed into auditory
images or ideas of objects and stored. From Wernicke’s area, auditory ideas flow through
a pathway, the arcuate fasciculus (from the Latin arc, for “bow,” and fasciculus, for “band
of tissue,” because the pathway arcs around the lateral fissure, as shown in Figure 1.7B).
The pathway leads to Broca’s area, where representations of speech movements are stored,
and may link brain regions related to intelligence (see Figure 16.17). To produce the
appropriate sounds, neural instructions are sent from Bro- ca’s area to muscles that control
mouth movements. According to Wernicke’s model, if the temporal lobe is damaged,
speech movements are preserved in Broca’s area, but the speech makes no sense because
the person cannot monitor words. Damage to Broca’s area produces a loss of speech
movements without the loss of sound images, and therefore Broca’s aphasia is not
accompanied by a loss of understanding.

Disconnection;

From his model, Wernicke also predicted a new language disorder but never saw such a
case. He suggested that, if the arcuate fibers connecting the two speech areas were cut,
disconnecting the areas but without inflicting damage on either one, a speech deficit that
Wernicke described as conduction aphasia would result. In this condition, speech sounds
and movements are retained, but speech is impaired because it cannot be conducted from
one region to the other. The patient would be unable to repeat what is heard. After
Wernicke’s prediction was subsequently confirmed, American neurologist Norman
Geschwind (1974) updated the speech model (Figure 1.7B) in what is now referred to as
the Wernicke- Geschwind model. Wernicke’s idea of disconnection offered investigators
a completely new way of viewing symptoms of brain damage by proposing that, although
differ- ent brain regions have different functions, they are interdependent: to work, they
must interact. Just as a washed-out bridge prevents traffic from moving from one side of
a river to the other and therefore prevents people from per- forming complex activities
such as commercial transactions or emergency re- sponse services, cutting connecting
pathways prevents two brain regions from communicating and performing complex
functions. Using this same reasoning, in 1892 French neurologist Joseph Dejer- ine (1849–
1917) described a case in which the loss of the ability to read (alexia, meaning “word
blindness,” from the Greek lexia, for “word”) re- sulted from a disconnection between the
 brain’s visual area and Wernicke’s area. Similarly, Wernicke’s student Hugo Liepmann
(1863–1925) showed that an inability to make sequences of movements (apraxia, from the
Greek praxis, for “movement”) results from the disconnection of motor areas from sensory
areas. Disconnection is important in neuropsychology, first because it predicts that
complex behaviors are built up in assembly-line fashion as information collected by
sensory systems enters the brain and traverses different structures before producing an
overt response. Second, disconnecting brain structures by cutting connecting pathways
can impair those structures in ways that resemble damage to the structures themselves.

Unit 4: Hemispheric asymmetry, cerebral commissurotomy

HEMISPHERIC ASYMMETRY

➢ The term hemispheric asymmetry refers to the differentiation in morphology and

physiology of the brain between the right and left hemispheres. The terms lateralization
and dominance refer to the differences in functional specialization between the two
hemispheres.
➢ Probably the function most associated with laterality is speech. Paul Broca first determined
that damage to the frontal operculum of the left hemisphere resulted in loss of speech
(aphasia), whereas the right hemisphere appeared to play little or no role in language
processing. Before Broca’s seminal finding, it was widely believed that the two
hemispheres were redundant in function. Broca’s discovery led to the proposition that
speech and language were properties of the left hemisphere.
➢ To say speech and language are lateralized to the left hemisphere denotes a functional
dominance of the left hemisphere over the right hemisphere for language processing.
Dominance does not mean that one hemisphere has complete or total responsibility for a
function, but rather plays a primary or major role in the support of a function. For example,
although there is significant empirical support for the lateralization of language to the left
hemisphere (for most people), research has demonstrated that the right hemisphere is, in
fact, able to perform a limited number of language functions such as understanding and
processing of rudimentary linguistic contents. It is unable, however, to process complex
linguistic information, and only rarely is able to communicate via speech. In addition, as
discussed later, the right hemisphere plays a major and complementary role in regulating
the nonverbal aspects of speech and language.
➢ Because of the decussation of the sensory and motor tracks from the brain to the spinal
cord, the right hemisphere shows greater involvement in the control of the left hand,
whereas the left hemisphere is associated with modulation of the right hand.
➢ Broca (1861) proposed that a person’s preferred handedness was opposite from the
hemisphere specialized for language.
➢ Subsequent research demonstrates that the relation of preferred handedness to hemispheric
lateralization is partially accurate when lateralization of right-handed individuals is
considered. For example, an MRI study (Springer et al., 1999) of 100 healthy right-handed
participants (48% female, 52% male) reported that 94% were left hemisphere dominant
for language, whereas 6% showed bilateral, roughly symmetric language representation.
None of the healthy participants were found to be right hemisphere dominant.
➢ Left-handed individuals are also likely to show left hemisphere lateralization for language,
although to a lesser extent than right-handed individuals.
➢ The determination of language laterality was made by having the participants perform
word generation tasks while undergoing Doppler ultrasonography (fTCD). Overall results
demonstrated a linear relation: the greater the righthandedness, the higher the incidence of
left hemisphere language dominance, and vice versa.
Hemispheric, anatomic and functional difference:

o Turning the brain to the lateral surface of the cerebral hemispheres, the Sylvian fissure
(which is normally the large fissure separating the frontal from the temporal and parietal
lobes) is steeper in the right hemisphere than in the left. This results in a larger parietal
and temporal area within the right hemisphere.
o It is reasonable to speculate that this allows higher level integration of visual, auditory,
and proprioceptive information in the more spatially oriented right hemisphere.
o From an anterior view, the frontal operculum (Broca’s area) of the right hemisphere has a
larger surface area, whereas the surface area of the left hemisphere is more pronounced in
the subcortical area. This may reflect differences in the language production abilities
between the two hemispheres.
o Within the superior temporal lobes is Heschl’s gyrus, and posterior to that is the planum
temporale. The Heschl’s gyrus (also known as the primary auditory cortex) of the right
hemisphere is often larger than that of
 the left hemisphere, since it frequently consists of two gyri. In the left hemisphere, the
planum temporale is larger than that of the right hemisphere. The structural differences
here may account for the functional differences in auditory processing between the two
hemispheres: Heschl’s gyrus may have greater functional responsibility for the nonspeech
aspects of language (pitch, tone, melody) and musical processing, whereas the planum
temporale plays a larger role in speech comprehension.
o In addition to visible structural differences, there are also differences in neuronal
architecture and neurochemistry, dependent on the side of the brain.
o The gray and white matter composition of the two hemispheres provides clues to the type
of processing associated with each hemisphere.
o The right hemisphere is heavier and contains more white matter than the left hemisphere.
In contrast, the left hemisphere is composed of greater gray matter and more modality-
specific sensory cortices.
o The composition of the left hemisphere suggests that it is more suited for single-modality
and intraregion or within-region processing.
o A greater convergence of sensory regions is evident in the right hemisphere, signaling an
increased representation of association regions. The increased association regions of the
right hemisphere allows for multimodal and interregional processing (e.g., the association
of the visual representation of an apple with its tactile representation).
o Because of these anatomic differences, it is hypothesized that the left hemisphere is better
suited for processing information that is linear, sequential, rule-governed, or conforms to
specific codes, such as language. In contrast, the right hemisphere appears more suited for
holistic or global processing of information; for example, determining the orientation of
one’s body in space (proprioceptive) or the spatial configuration and orientation of objects
requires holistic or simultaneous processing of representative sensory input.
o Dean and Reynolds (1997) have summarized the functions and contents that are
considered to show lateralization (Table 6.2).
 o The right hemisphere appears to show superiority to the left in “veridical processing”
(accuracy of information representation), whereas the left tends to process information from
an elaborative or explanatory perspective (interpretation and hypothesis generating),
regardless of whether the content is verbal, facial, or abstractfigural.

NEUROPSYCHOLOGICAL AND BEHAVIORAL CEREBRAL DIFFERENCES:

• Except for smell and taste, sensory processing is funneled from the site of the sensory
stimulation to the contralateral, or opposite, hemisphere.
• For example, the somatosensory strip of the parietal lobe of the left hemisphere processes
objects placed in the right hand. The primary occipital cortices of the left and right
hemispheres are involved in the processing of information in the right and left visual fields,
respectively. These two senses are completely crossed, because the neural tracts project
only to one hemisphere, opposite from the body site of stimulation.
• Audition is only partially crossed. The left hemisphere processes the majority of
information presented to the right ear, whereas the right hemisphere processes a smaller
portion of this auditory input.
• Olfactory information, which evolutionarily represents a more primitive sense, projects to
the same (ipsilateral) hemisphere from each nostril; the right nostril is processed via the
right olfactory bulb. Similarly, each hemisphere receives taste sensations from its
respective side (ipsilateral) of the tongue.
• As a general way of conceptualizing the differences between the hemispheres, the side that
is dominant for verbal abilities (often the left) is usually more proficient in speech
production and understanding. It is facile in manipulating the symbols of language, such
as letters, words, and numbers. Both hemispheres are comparable in processing sound,
with the left hemisphere (temporal region) responding selectively to meaningful language.
• The right hemisphere is more adept at processing the melodic, or prosodic, aspects of
sound. Furthermore, the right hemisphere holds the advantage for visuospatial
transformations, analysis of complex visual patterns, and emotional processing.
• Regarding emotional processing, the right hemisphere is thought to be more adept at
reading emotions when expressed as gestures, tonal inflections, and facial expressions.
When the right hemisphere is damaged, patients often speak in a monotone, fail to
understand the emotional expressions of others, miss the gist of conversations, have
 problems expressing emotions nonverbally and verbally, and are limited in their
understanding of humor and sarcasm.
• Moreover, the right hemisphere, particularly the prefrontal cortex, exhibits preferential
control of attentional functions such as arousal, sustained and selective attention, response
inhibition, and self-monitoring. Although the left hemisphere is less involved in attentional
functions, it does play a significant role in divided attention.

CEREBRAL COMMISSUROTOMY

Epileptic seizures may begin in a restricted region of one hemisphere and spread through
the fibers of the corpus callosum (the commissure) to the homologous location in the opposite
hemisphere. To prevent the spread of a seizure when medication has failed to impose control,
commissurotomy, the surgical procedure of disconnecting the two hemispheres by cutting
the 200 million nerve fibers of the corpus callosum, was performed first in the early 1940s
by William Van Wagnen, an American neurosurgeon. The procedure’s therapeutic outcome
initially appeared too variable, and commisurotomy was abandoned until the 1960s, when
research with monkeys and cats by Ron Myers and by Roger Sperry led neurologists to
reconsider it.

After sectioning, the two hemispheres are independent: each receives sensory input from all
sensory systems, and each can control the body’s muscles, but the two hemispheres can no
longer communicate. Because functions in these separate cortexes, or split brains, are thus
isolated, sensory information can be presented to one hemisphere, and its function can be studied
without the other hemisphere having access to the information. Input from the left side of the
world (the left visual field) goes to the right hemisphere, whereas input from the right side of the
world (the right visual field) goes to the left hemisphere. A connection through the corpus
callosum joins the two sides. With the corpus callosum severed, the brain cannot join the different
views of the left and right hemispheres together. When the left hemisphere of a split-brain patient
has access to information, it can initiate speech and hence communicate about the information.
The right hemisphere apparently has reasonably good recognition abilities but cannot initiate
speech, because it lacks access to the speech mechanisms of the left hemisphere.

Commissurotomy, the surgical severing of the cerebral commissures, is an elective treatment for
intractable epilepsy in cases in which medication proves ineffective. Seizures may begin in a
restricted region of one hemisphere (most often in the temporal lobe), then spread through the
 fibers of the corpus callosum or anterior commissure to the homologous location in the opposite
hemisphere. As a result of commissurotomy, each hemisphere retains fibers that allow it to see
only the opposite side of the visual world. Likewise, each hemisphere predominantly receives
information from the opposite side of the body and controls movements on the opposite
side of the body.

The surgery also isolates speech in persons with lateralized speech. Consequently, the dominant
hemisphere (usually the left) is able to speak, and the nondominant hemisphere is not. About a
year or so is required for recovery from the surgical trauma. Within 2 years, the typical
commissurotomy patient is able to return to school or work. A standard medical examination
would not reveal anything unusual in these split-brain patients’ behavior, and their scores on
standardized tests are at normal. The patients’ everyday behavior appears similar to that of
typical
“ unified” people. Specific tests, such as those Sperry and his coworkers obtained with their
split-brain patients (e.g., Sperry, 1974), can show differences between the functioning of split-
brain patients and that of people with intact cerebral connections. In the split brain, each
hemisphere can be shown to have its own sensations, percepts, thoughts, and memories that are
not accessible to the other hemisphere. The usual testing procedures include presenting stimuli
only to one hemisphere then testing each hemisphere for what transpired.
Odors presented to one nostril cannot be identified by the other, objects seen in one visual
field cannot be recognized in the other, and so on. Although the hemispheres function
independently, they both do so at a high level, even in language skills. The non-dominant
hemisphere, although unable to speak, can understand instructions, read written words, match
pictures to words, and match written to spoken words. Non-dominant language ability is best
for nouns and poorest for verbs.
The non-dominant hemisphere performs in a superior fashion on a variety of spatial tasks,
including copying designs, reading facial expressions, and fitting forms into molds. The
nondominant hemisphere also has a concept of self and can recognize and identify social
relations and pictures of the person in a social relation as well as pictures of family members,
acquaintances, pets and belongings, and historical and social figures. Each hemisphere also has a
general awareness of body states such as hunger and fatigue.
 MODULE 2: DISRUPTION OF HIGHER CEREBRAL FUNCTIONS

Unit-1 Common Neurological Disorders

1. Cerebral Trauma
 Functions of the brain are disturbed by physical injury and effects of penetrating wounds on the
brain resulting from shrapnels.
 Craniocerebral injury rapid acceleration and deceleration of the head is another type of injury
 Even without fracturing the skull, a wide variety of lesions can occur:
-Generalised lesions scattered across the brain with or without localized damage such as contusion,
laceration or haemorrhage.
 Cerebral damage can be classified as primary or secondary in nature:
-Primary lesions are associated with trauma itself and its principal forms are- contusion, laceration
and haemorrhage.
-Contusion and laceration cannot be distinguished on clinical grounds.
- These can be a mixture of all 3 as well.
 Secondary lesions- arises from reduction of blood supply (ischemia), reduction of oxygen to the
tissues (anoxia), brain swelling (oedema) and intracranial haemorrhage.
Mechanism and Sites of Cerebral Contusion
 2 important descriptive terms used are:
Coup injuries referring to damage beneath the site of impact
Contre-coup injuries refer to lesions away from the site by a considerable distance.
 Courville (1942) observes that greatest zone of brain contusion following brain injury was not
invariably opposite to the impact.
 Identical lesions of subfrontal and anterior temporal regions-results from the contact of either
the frontal region or occipital region of the moving head-And yet there are no gross coup or
contra coup lesions in the occipital region.
 This indicates that anatomic relation of the brain and portion of the skull proximate to it is
responsible for the nature and distribution of the lesion.
 In motor accidents with high velocity deceleration, damage to anterior portion of temporal lobe
and sometimes the frontal lobe is so great that it produces subdural haemorrhage brain
contusion, laceration and intracerebral bleeding that has been termed as the exploded pole
  Understanding cerebral contusion explains why sometimes- psychological deficits occurs in
brain injured patients-which suggests damage to localised areas far from impact. E.g. Frontal
lobe signs such as uninhibited behaviours, lack of planned initiative etc.
2. Concussion

 Also known as impairment

consciousness

➢ Simple Concussion:

• Brief clouding of consciousness or a temporary loss.

• Usually caused by injury to the brain stem reticular formation
• Patient cannot remember the incident causing the concussion and has a loss of memory for
events just preceding it, called retrograde amnesia
• Memory loss for the period subsequent to the injury termed as anterograde amnesia

Measures of Severity

1. COMA: - Employing the length of coma as a measure of severity against which clinical and social
sequelae- had difficulties due to the uncertainty of defining coma.

-Widespread adoption of Glasgow Coma Scale chart- standardized to measure eye opening motor
response and verbal response.

2. Post-Traumatic Amnesia (PTA)- Introduced by Russel and Nathen (1946)

-The length of the time from injury to the time the patient became aware that he had regained
consciousness.
-Corresponds to the time when the patient begins to retain a stable record of ongoing events.

-Russel and Nathan (1946) combined their definition with Russel and Smith (1961) resulting in a
definition of concussion as the combined period consisting of the length of unconsciousness with the
period when the patient is awake and responding but, not consolidating memories i.e, coma plus the
period of anterograde amnesia.

-Artiola, Fortuny et al (1980)- Westmead Scale used to measure PTA

3. Traumatic Haemorrhage:

 Extensive haemorrhage occurs from the laceration of blood vessels inside the skull. For
example, a fracture of the skull may tear the middle meningeal artery- which by bleeding under
considerable pressure into the space outside the covering of the brain-greatly compresses the
cerebral hemispheres and later the basal portions of the brain (this extradural hematoma is
considered as neurosurgical emergency).
 Bleeding from blood vessels beneath the dura matter produces a trivial injury that the patient
does not even recall.
 The subdural hematoma produces changes that develop over days or even weeks after the
injury-resembles the clinical picture of dementia with loss of concentration, episodes of
confusion ad memory loss.

Unit 2: CEREBROVASCULAR DISORDERS

Any disruption of brain function arising from some pathological condition related to the blood vessels.
These produce an array of disorders of great complexity.

Vascular pathology may take many forms, E.g.

• lesions of the walls of the blood vessels themselves in the form of deposited material (atheroma) with
or without ulceration
• rupture of the vessel wall itself
• narrowing (stenosis), or total occlusion of the lumen from thickening of the vessel wall
• the presence of an obstructing clot (thrombus) or embolus
• changes in the characteristics of the blood itself.
The term stroke itself is not clearly defined and is not a precise medical term. A more technical term
for stroke is cerebrovascular accident (CVA), which describes a heterogeneous group of vascular
disorders that result in brain injury. The brain’s blood vessels are damaged, which can decrease blood
flow within and to the brain. In simple terms, stroke “suffocates” brain tissue and often produces an
area of dead or dying brain tissue. A stroke always occurs in the brain and is the most common type
of cerebrovascular disease. Thus, CVA or stroke is a common label assigned to clinical syndromes
that are caused by blockage in blood supply or by bleeding in the brain. Stroke can result from a wide
variety of different vascular diseases, but not all vascular disorders produce stroke. Although the onset
of symptoms is rapid (hence the term stroke), the full development of the clinical picture may take an
appreciable time, sometimes hours, depending on the rate of the bleed and its final cessation
(Bannister, 1992).

Stroke can produce an array of disorders of great complexity. Most strokes occur, or are localized, in
only one of the cerebral hemispheres, although in some instances, multiple strokes or one major
subcortical stroke affects the entire brain. In general, the deficits directly relate to the location or the
hemisphere where the stroke occurs.

Victims may have to contend with these three serious consequences of stroke:

1. Disrupted blood supply decreases oxygenation, as in hypoxia or anoxia, of the involved brain
tissue.

2. Related to bleeding (or haemorrhage), a space-occupying mass or pocket of blood often develops,
which may press on nearby brain structures, affecting their integrity. As a result, intracranial pressure
(ICP) may suddenly increase.

3. When blood spills out of the artery and into brain plasma, many toxins in the blood can interfere
with normal brain metabolism. In an intact vascular system, the blood–brain barrier (BBB) protects
the surrrounding tissue from any toxic properties contained in blood, but exposed blood outside of the
artery irritates brain tissue.

While the types of cerebrovascular disease are numerous, most cases are due to cerebral ischemia
(deprivation of blood supply) or to hemorrhage. Ischaemia may be transient with symptoms that
recover. Any prolonged ischaemia will lead to death of tissue or infarction. The most common cause
of infarction is thromboembolism and this accounts for two-thirds of all cases of cerebrovascular
disability. Some 15-25% cases are caused by intracerebral or subarachnoid hemorrhage, while the
remaining 5-10% result from less common causes.

1. Cerebral ischaemia

Transient ischaemic attacks (TIAs)- refers to recurrent attacks of short-lived local neurological deficit
produced by temporary ischaemia. Recovery should take place within 24 hours but is often complete
within a much shorter time. Although, the episodes take many forms they are often rather similar, even
stereotyped, in the one individual. Rudolf Virchow, a German physician and the founder of German
medicine, coined the term ischemia (derived from Greek ischein, meaning “to hold in check,” and
haima, meaning “blood”); literally, ischemia means “a holding in check of the flow of blood” to refer
to the temporary disruption of blood flow.
The patient experiences sudden loss of neurological function. This may be loss of power, which often
begins distally, but may progress to affect the whole limb or an entire side. There may be sudden
sensory loss, particularly loss of vision in one eye (fleeting blindness), or loss of speech, memory, or
the functions referable to the cerebral hemisphere territories supplied by the internal carotid system. It
is unusual for the brain and the eye to be affected simultaneously in one attack. When the
vertebrobasilar system is compromised there may be symptoms such as ataxia and vertigo, signalling
dysfunction in the cerebellum or brain stem. The attacks vary from infrequent to frequent. During the
attack the neurological signs will be indistinguishable from a developing infarct, but examination
between the episodes will be normal.

In transient ishcaemic attacks, it is generally assumed that the ischaemia has occurred without actual
death of neurons, though this may not necessarily be the case, and the apparent absence of findings
may represent the insensitivity of functional measures as well as the relatively silent location of the
affected territory. TIAs most commonly involve the internal carotid, middle cerebral, or the
vertebralbasilar arteries. Clinically, neuropsychologists divide the deficits into the anterior circulation
involving the carotid system and those affecting the posterior circulation involving the vertebral-
basilar system. If the ischemic attack affects anterior circulation, the deficits manifested will most
likely relate to brief clumsiness or weakness of a limb, dysarthria, or aphasia. If the attack affects the
posterior circulation, deficits may include dizziness, neglect, double vision, and numbness or weakness
of the extremities.
Surgical treatment
Two major types of surgical procedure have been devised to prevent strokes and ischaemic attacks by
reconstituting the arterial blood flow. The most common is the operation of endarterectomy, which
aims at correction of stenosis, removal of atheromatous plaques, or removal of an organized thombus
and other conditions compromising the circulation. Its is sometimes necessary to remove totally a
diseased segment of artery and replace it with a vein graft. The operations are carried out at the
predilection for atheroma formation e.g, at the orgin of the internal carotid artery. The indication for
operation is usually the presence of transient ischaemic attacks in the territory distal to a radiologically
demonstrated high grade stenotic or ulcerated lesion.
2. Cerebral infarction

When ischemia is severe enough to kill the nerve cell, neurologists consider the neuron infarcted.
Infarctions result from an inadequate blood supply to an area of the brain, causing tissue death or
necrosis from the lack of oxygen. This event most often relates to obstruction of the local vascular
circulation by blockage or occlusion of a vessel, stopping blood flow. Occlusions are typically caused
by either a blood clot or a fatty deposit lodged in a vessel. The area of the brain in which this occlusion
occurs depends on clot size. Occlusion can happen to the brain as a whole, as in severe heart failure;
in one of the major arteries or their branches supplying blood to the brain; or in a small capillary.
Cerebral infarction is most likely the result of a thrombotic or an embolic vascular occlusion in blood
flow.

The basic pathological process

The most common basic pathological process in infarction is atherosclerosis. This is not a uniform
process but one which affects certain parts of the artertial system more than others. The deposition of
materials, mostly cholesterol, causes plaques which narrow the artery and thus restrict the flow. This
mat progress to complete occlusion of the affected vessel. Whether the infarction occurs depends upon
whether there is a collateral source of supply for the affected region. In general, however, the size of
an infarct depends to a large extent on the size of the vessel occluded- this may range from the minute
to massive death of a large part of the hemisphere from sudden occlusion of an internal carotid artery.

There are certain sites of predilection in the formation of atheromatous plaques, mostly where arteries
branch or bifurcate. The most common sites are the origin of the internal carotid artery, the upper end
of the vertebrals and the lower portion of the basilar, the stem of the middle cerebral, and the posterior
cerebral, though other vessels are also commonly affected. The plaques may ulcerate and the debris
which collects, e.g, platelets, fibrin and cholesterol, may detach in the form of emboli and travel further
afield to block smaller vessels.

The most common cause of cerebral embolism is thrombi forming in the heart because of arrhythmias
or myocardial infarction. The term embolism (derived from Greek embolos, meaning “plug” or “wedge”)
refers to a blood clot that has travelled from one part of the body to another. Sometimes a piece of plaque
originally formed in the heart can “break” off into the blood circulation and travel to the brain. If the
blockage is not relieved rapidly, the brain area served by that artery dies of infarction.
A dramatic embolic phenomenon is amaurosis fugax. The term literally means ‘fleeting blindness’ and
the condition is most commonly caused by emboli blocking retinal arterioles. Embolism are often (up
to 33%) associated with a condition known as atrial fibrillation, an arrhythmia of the heart. When a
traveling blood clot or embolism lodges in a distal intracranial branch of a blood vessel, it may block
blood flow to specific parts of the brain, so a cerebral infarction occurs.
On the other hand, Thrombosis is a type of occlusion in which a clot or thrombus forms in an artery
and obstructs blood flow at the site of its formation. This is the most common form of stroke and
accounts for approximately 65% of all CVAs.

3. Cerebral hemorrhage.

Hemorrhage (derived from Greek haima, meaning “blood,” and regnynai, meaning “to burst forth”)
results from rupture of a blood vessel causing heavy spilling of blood into cerebral tissue. Bleeding
within the cranial cavity may result from a large number of causes, however three conditions account
for the majority of cases. In each condition the severity may vary from small, almost symptomless
bleed to a massive hemorrhage leading to sudden death.

• Hypertensive intracerebral hemorrhage

This is the most common form of haemorrhage, with extraversion, bursting out of blood into the
substance or parenchyma of the cerebral hemisphere. The bleeding destroys brain tissue and, if it
continues, causes pressure effects on neighbouring tissue by compression or displacement. Large
hemorrhages may so displace vital centres that they lead to death. Less serious pressure may disrupt
the function or adjacent or nearby tissue without destroying it and this accounts for the partial, though
often considerable, recovery of function after the acute stages. While the onset of symptoms is rapid,
hence the term ‘stroke’, the full development of the clinical picture may take an appreciable time,
sometimes hours, depending on the rate of bleeding and its final cessation. When bleeding stops there
is no recurrence from the same sie, a situation unlike that of bleeding from aneurysms. While the
bleeding may commence in the brain’s substance, it breaks into the ventricular system and thus into
the cerebrospinal fluid in a high proportion of cases.
The brunt of symptomology with hemorrhage tends to be on neurological rather than behavioural
features, since the cortex is often relatively spared. Sites of primary hemorrhage in decreasing order of
frequency are the basal ganglia, thalamus, cerebellar hemispheres, pons and subcortical areas.
Hemorrhages arise mainly from the penetrating branches of the middle cerebral, posterior cerebral and
basilar arteries.
• Ruptures aneurysm
These thin-walled protrusions are most common on the vessels which form the arterial circle (circle
of willis) or their major branches. They are assumed to be developmental defects in the arterial walls
and are prone to rupture. Over 90% are found on vessels of the internal carotid system, only a few on
the vertebrobasilar system. The most frequent sites of rupture are the anterior communicating region
 and the middle cerebral bifurcation, accounting for nearly half the cases. Sometimes the aneurysms
are multiple.
In rupturing, blood may be spurted into the subarchnoid space or into the adjacent brain substance,
producing an intracerebral haematoma. Bleeding into the cerebral substance is particularly prone to
occur at the two most common locations mentioned. The territories of vessels in the area often show
infarction- this is possibly related to arterial vasospasm since at the autopsy the vessles may be patent.
The clinical symptoms and signs are those related to blood under pressure in the subarachnoid space.
Usually excruciating headache and collapse, followed in survivors by those of local disruption of
functions by local pressure from extracerebral blood clot, intracerebral haemorrhage and infarction.
• Ruptured arteriovenous malformation (AVM)
These developmental malformations vary from a small localized abnormality of a few milimetres to a
large mass of vessels occupying considerable space, often in the form of a wedge extending from the
cortex to the ventricle. The blood vessels forming the mass interposed between the feeding arteries
and the draining veins are pathologically thin-walled and liable to rupture. Rupture of the larger
malformations may produce intracerebral as well as subarachnoid bleeding. Like sccular aneurysms,
AVMs have a tendency to recurrent bleeding. Hydrocephalus is a compilation in some cases.
Symptoms and signs are thus extremely varied.

Unit 3: Aphasia, Agnosia, Apraxia, Amnesia

1. APHASIA

• Aphasia is a disturbance of language usage or comprehension. It may impair the power to speak,
write, read, gesture, or to comprehend spoken, written, or gestured language. It is a disturbance
connecting speaking to thinking, and thus is differentiated from purely mechanical disorders of
speech such as dysarthria or speech apraxia caused by paralysis or incoordination of the
musculature of the mouth or vocal apparatus.
• Aphasic disorders also do not include pure mutism, that is, disturbances of language caused by
severe intellectual impairment or loss of sensory input (such as deafness or inability to see words).
• Aphasias are most frequently caused by vascular disorders such as stroke or by tumor or brain
trauma. Although cortical damage to the frontal and temporal areas of the left hemisphere causes
most aphasias, damage to subcortical structures of the basal ganglia (specifically the corpus
striatum) and the thalamus has also produced aphasia.
• The two major types of aphasia are commonly referred to as Broca’s aphasia and Wernicke’s
aphasia. Other classifications, subtypes, and combinations exist that may contain slightly different
features or a mixture of some features of these two types (see Table 8.1). In seeing aphasia patients,
it is quickly evident that few patients fit neatly into one classification or the other. This is because
brain lesions and pre-existing brain anatomy for language varies among individuals, most notably
between men and women. Many clinicians find it most useful to describe the features of the aphasia
in behavioral terms, describing aphasia according to degree of fluency and the nature of the
expressive and receptive problems involved.
• Fluent aphasia is characterized by fluent spontaneous speech with normal articulation and rhythm,
or fluency in the repetition of words, phrases, or sentences.
• Non fluent aphasia is difficulty in the flow of articulation, so that speech becomes broken or
halting.
• Expressive aphasia is a disorder of speech output.
• Receptive aphasia implies a difficulty in auditory comprehension. Often, patients experience not
only a loss of spoken language but also a loss of written language and reading comprehension.
- Writing is one of the most complex language abilities. If it is disturbed as a result of impairment
of the limb to produce letters and words, the disturbance is not thought to be a disorder of language.
Rather, words, letters, or numbers may appear foreign or incomprehensible because of an inability to
recall the form of letters.
- Attempts at writing may be filled with real or imagined letters that make no sense to others. With
time and training, the person may be able to understand simple written language (for example, single
words such as bath or food ) but not more complex written language, such as sentences or paragraphs.
The extent of the recovery in this and other areas largely depends on the degree of damage.
- Because reading comprehension is based on prior mastery of auditory language, deficits in reading
(alexia) often accompany deficits in auditory comprehension. As with auditory defects, impairment in
visual comprehension may involve a deficit in recognizing individual letters or words as being letters or
words, or an impairment in attaching the correct meaning to the symbols written on a page. Deficits in
writing are most often associated with lesions to the angular gyrus, a cortical association area that
provides cross modal integration of visual, tactile, and verbal information. Reading deficits (alexia) are
frequently related to lesions of the left fusiform and lingual areas.
 Broca’s Aphasia

• Broca’s aphasia is an expressive, non fluent aphasia characterized by difficulties in speech production
but relatively adequate auditory verbal comprehension, as evidenced by the ability to follow spoken
commands (such as, “Point to the cup”). Many forms of disorders of production exist, which can range
from an inability to form words to an inability to place words together to form a spoken or written
sentence.
• In simple expressive aphasia, the person knows what he or she wants to say but cannot find the words
to say it. It is like continually experiencing a situation in which the word or words are on the tip of the
tongue but are not quite connected to thought.
• In mild cases of anomia, or word-finding difficulties, only a word or two here and there is lost, and
the communication can proceed quite normally. In more severe cases, most or all words can be lost.
This problem in word finding is one that virtually all expressive aphasics suffer. Even when the person
produces words, he or she takes longer than normal to do so. Difficulty in finding a word often results
in the person’s deliberately “talking around” a word that approximates the intended idea when he or
she cannot find the intended word. For example, an aphasic might say, “I looked through that long
pipe thing at the stars” (that is, a telescope). Aphasics may also make phonetic or like-sounding errors,
such as “I looked through that telephone at the stars,” or semantic meaning–related errors, such as “I
looked through that barometer at the stars.”
• Another problem experienced by many Broca’s aphasics is one of articulation, or the ability to form
phonetic sounds of vowels and consonants, which then are placed in different combinations to form
words and sentences. People with severe deficits in articulating words often cannot produce simple
sounds, even by imitation. If the deficit in articulation is related to a motor impairment of the mouth,
tongue, larynx, or pharynx, then it is not aphasia. The aphasic impairment is marked by the person’s
confusion or deficit in choosing the desired sound from all those available in his or her repertoire.
• This sometimes results in unintended strange-sounding syllables, words, or phrases (phonemic
paraphasias) during the effort to speak, or in noises being produced rather than language. Those with
Broca’s aphasia may also show poor pronunciation and inappropriate speech rhythm, manifested by
dysarthria, stuttering, and effortful speech.
• Broca’s aphasia most commonly occurs with lesions to the frontal operculum, but typically also
includes lesions to the motor cortex (precentral gyrus or motor strip) and underlying white matter and
subcortical structures of the basal ganglia. Aphasia appears to resolve quickly if the frontal operculum
is the only structure involved, but it tends to be more persistent the more surrounding structures are
damaged (Bradshaw & Mattingly, 1995).
Wernicke’s Aphasia

• Wernicke’s aphasia is a receptive, fluent aphasia. This implies reduced comprehension of spoken
language with the continued ability to produce speech.Wernicke’s aphasics, in addition to not
understanding what others say, may not be able to understand what they themselves are saying.
This problem contributes to speaking in the form of a word salad of unconnected words and word
sounds. This feature of Wernicke’s aphasia is a deficit in putting words together in proper
grammatical and syntactical form. This condition, more formally known as paragrammatism or
extended paraphasia, refers to running speech that is logically incoherent, often sounding like an
exotic foreign language. That is, the speech of a person with Wernicke’s aphasia flows forth
without hesitation and has appropriate intonation. Appropriate social interaction, such as speaking
in turn and gesturing appropriately, remains intact. However, the words and phrases spoken are
meaningless.
• Wernicke’s aphasics usually do not realize their spoken language is meaningless to others
(anosognosia for speech). It is as if they know exactly what is to be communicated, but their
delivery is incoherent.

• In mild cases of Wernicke’s aphasia, only a word or two here and there sounds garbled or
incomprehensible. In this case, communication can generally proceed because the person is able
to grasp the essence of the intention based on the context within which the communication takes
place. With moderate disability, the patient can understand part, but not all, of the communication.
• In severe cases, the patient may experience most or all speech as if it were nonsense syllables or a
foreign language. It is not uncommon for others to misattribute behavior problems to people with
receptive aphasia. Sometimes noncompliance is actually caused by the fact that the patient has
misunderstood the communication in the first place. Thus, a patient may act as though a word was
not heard at all (so called word deafness) or as though only fragments of the word were heard.
• The most common defect, however, lies not in failing to recognize that a word was spoken, but
rather in failing to attach meaning to the word. In some aphasics, comprehension of individual
words is intact, but grammatical constructions are not.
• Damage in Wernicke’s aphasia includes the secondary auditory cortex (Wernicke’s area) and some
involvement of surrounding structures. These often include the supramarginal and angular gyri and
portions of the middle temporal gyrus.
Other Aphasia Subtypes

The classification of aphasias into receptive and expressive is a useful didactic tool, but many people
with left hemisphere lesions have a combination of both symptoms, because of damage to the left
middle cerebral artery, which serves both expressive and receptive areas.

Conduction Aphasia—The behavioral hallmark of conduction aphasia is a problem in repeating what

others say. This problem, may not become apparent except on formal testing. In ordinary conversation,
expressive speech is fluent but marked with phonemic paraphasias, or errors of word usage of
similar-sounding words (such as using the word bark for tarp).

- Comprehension is relatively well preserved but may suffer from minor errors. Reading aloud and
writing are frequently impaired.

- Neuroanatomically, conduction aphasia is a result of separation of Broca’s area from Wernicke’s

area by damage of the arcuate fasciculus, the connecting white matter fibers between the two areas.
The damage may also include lesions to the posterior portions of the lateral fissure responsible for
aspects of reading and writing, specifically the supramarginal and angular gyri.

Transcortical Motor Aphasia—Clinicians can recognize transcortical motor aphasia by the patient’s
halting, nonfluent spontaneous speech; oddly, however, speech becomes fluent if the person merely
repeats what another says. In many respects, except for the differences in repetition ability, this deficit
resembles Broca’s aphasia. Speech comprehension is unimpaired and writing may also suffer. Reading
comprehension, however, is generally intact. Lesions to the area anterior or superior to Broca’s area
are associated with this aphasia type.

Transcortical Sensory Aphasia—Severe speech comprehension deficit marks transcortical sensory

aphasia. Interestingly, despite being unable to comprehend speech, such aphasics can adequately repeat
phrases and sentences presented to them. Lesions in the angular gyrus are the most likely culprits for
this aphasia; thus, reading and writing are also affected.
Anomic Aphasia—A problem in word finding, or anomia, is the primary, and often only, difficulty
in anomic aphasia. This aphasia is a frequent result of widespread brain impairment caused by
conditions such as traumatic brain injury and dementias such as Alzheimer’s disease. As people age,
an annoying concomitant often reported is a difficulty in remembering people’s names or being unable
to retrieve a word that is just on the “tip of the tongue.” The act of “word finding” seems to be one of
the most easily affected aspects of expressive language.It is difficult, therefore, to distinguish what
might be a temporary word- finding difficulty and a true aphasia.

Global Aphasia—Global aphasia is the most devastating of the aphasia subtypes because of its
profound effect across all areas of speech functioning. There is marked disability in speech
production, as well as speech comprehension. Reading, writing, and repetition are also impaired.
This aphasia is caused by a massive lesion encompassing major portions of the left hemisphere.

2. AGNOSIA
- Agnosia, the inability to recognize the form and/or function of objects and people, occurs in every
sensory domain. For example, tactile agnosia, also called astereognosis, is an inability to recognize
objects by touch; for instance, failing to recognize a quarter or a pen held in the hand.

- Agnosia is possible in every sensory domain and can sometimes result in odd behavior. Agnosia
is not a single sensory phenomenon. It may strike any sensory domain, but the most commonly studied
instances affect the somatosensory visual and auditory senses. Olfactory and taste agnosias have not
been researched extensively. Agnosias, in general, are relatively rare, but they have received
considerable attention in the neuropsychological literature, perhaps because they so strikingly alter
consciousness.
 Examples of somatosensory dysfunction

i. Astereognosis/tactile agnosia: inability to recognize an object by touch ii.

Finger agnosia: inability to recognize or orient to one’s own fingers

- Agnosia is derived from the Greek, gnosis, meaning “absence of knowing” and can occur in
any sensory domain. In the modality of vision, people with visual object agnosia may fail to recognize
objects at all, or in milder cases, confuse objects that they observe from different angles or in different
lighting conditions.

- The term prosopagnosia refers to the inability to recognize people by their faces, even though
the person can often recognize people by other means such as gait or tone of voice. People with visual
agnosias can see. The disorder is less a pure sensory disorder than a higher perceptual disorder of
“knowing.”

Eg: In The Man Who Mistook His Wife for a Hat, Oliver Sacks describes the affliction of Dr. P, a
music teacher who can no longer recognize objects or people by sight. Presented with a red rose, Dr.
P “took it like a botanist or morphologist given a specimen, not like a person given a flower. ‘About
six inches in length,’ he commented. ‘A convoluted red form with a linear green attachment.’” Dr. P
was completely unable to name what he had in his hand until it was suggested to him to smell it.
“‘Beautiful!’ he exclaimed. ‘An early rose. What a heavenly smell!’ He started to hum [the German
tune] “Die Rose, die Lilie. . . .” (Sacks, 1987, pp. 13–14). Dr. P’s affliction was that he was visually
unaware of the totality or gestalt of objects. He could see and identify form and color but could not
combine these aspects into a higher sense of meaning that is a rose. His only visual reality was a
mechanistic identification of features. This is typical of how visual agnosia primarily involves the
processes necessary for object recognition or object meaning while leaving intact elementary visual
processes. Also, Dr. P’s agnosia, as is usually the case, was modality specific. Although his visual
knowing was impaired, a higher sense of knowing was available through sense of smell. Dr. P also had
no problem in recognizing people by their voices.

-Apperceptive visual agnosia : The essential difficulty in apperceptive visual agnosia is object
perception, or the inability to combine the individual aspects of visual information such as line, shape,
color, and form together to form a “whole” percept. They seem to see in bits and pieces, like the
proverbial blind men feeling the elephant. Their brains are not synthesizing the entire picture.
-

- At first glance, those individuals, like Dr. P., with the apperceptive form of object agnosia may
be thought blind, because they tend to take no apparent notice of objects and people in their vicinity.
But on closer examination, their sensory functions are clearly intact. Many people with this condition
are aware that they can indeed see, but they have a problem correctly perceiving things. Curiously,
others with apperceptive agnosia are strangely unaware of their condition. Only watching for a period
of time might you catch them stepping over or avoiding objects. Awareness in this case is not an
either/or phenomenon.

- Apperceptive agnosics may appear to disregard or show no concern for their problem until
neuropsychological testing reveals it to them. Apperceptive agnosics also have difficulty copying
objects. Because they only “see” pieces, their drawings are likely to appear as a set of unconnected
fragments focusing on the details rather than on the entire gestalt of the object.

- The most common site of damage in apperceptive agnosia is the parieto-occipital area of the
right hemisphere. Sudden insults to the brain are the most common cause, often from carbon monoxide
poisoning, mercury intoxication, cardiac arrest, or stroke. In these cases, apperceptive agnosia does
not usually occur in isolation without other visuospatial impairment, because these brain insults are
likely to affect large areas of the cortex. Some cases of apperceptive agnosia are caused by bilateral
cortical atrophy.

- If both hemispheres are involved, then the patient may have Balint’s syndrome, which
includes visual agnosia together with other visuospatial difficulties such as misreaching and left-sided
neglect.

- Associative visual agnosia: Associative visual agnosics have difficulty to varying degrees in
assigning meaning to an object. Even though they can, for instance, recognize differences in form
between pictures of a pair of scissors and a paper punch by matching the scissors to a like pair in a
display of office objects (with which an apperceptive agnosic would have difficulty), they have lost
the link between the visual percept and the semantic meaning. In both cases, if shown a pair of scissors,
neither the apperceptive nor the associative agnosic can correctly name “scissors.” But although the
associative agnosic can pick out a pair of scissors, she or he shows difficulties not only in naming but
in explaining or demonstrating the use for scissors.

- Associative agnosia is differentiated behaviorally from apperceptive agnosia in that the primary
difficulty is a loss of knowledge of the semantic meaning of objects. Conceptually, the person can
“recognize” objects at a perceptual level by picking them out, or correctly copying them, but perception
breaks down at a higher level of meaning. For example, some people have little apperceptive difficulty
and can draw or copy pictures of objects in great detail but cannot name them (for example, see Rubens
& Benson, 1971).
The perceptual mistakes of the apperceptive agnosic are likely to show inability to recognize the
whole, whereas the perceptual mistakes of the associative agnosic may show problems of either
recognition of the whole or of the details of an object.

- The research on the neurocorrelates of associative agnosia is confusing. A lateralized left

hemisphere parieto occipital lesion may be enough to cause an associative agnosia, although it can
also occur in the presence of a unilateral right occipital lesion. Indeed, a number of structural areas
may produce associative agnosia.

- Farah (1990) suggests the variety of sites that produce associative agnosia may lead to
heterogeneous perceptual impairments. Because assigning meaning is such a high-level cortical
process, different lesion sites producing a similar effect also speaks to the complexity of the perceptual-
meaning system. In general, the left hemisphere assigns meaning, whereas the right hemisphere
governs the global aspects of perceptual integration.

- Many patients show both apperceptive and associative aspects to their visual agnosia. For
example, one artist who experienced a stroke resulting in bilateral medial occipital damage could name
some objects but not others. Those he could name he could also draw well. But those he did not
recognize, he could only mechanistically copy, feature by feature, first a square, then a circle, then
connecting lines, without any inkling of what they represented (Wapner, Judd, & Gardner, 1978). In
summary, the differentiation between apperceptive and associative agnosia is useful for descriptive
and conceptual understanding, but it does not correspond to strict anatomic correlates that can be
readily differentiated or dissociated.

3. APRAXIA
- In general, the term apraxia refers to an inability to perform purposeful sequences of motor
behaviors. Although basic motor skills may be intact, the patient may be unable to perform even
overlearned motor sequences. Apraxia is the main type of disorder under the category of problems of
how to act.

- Strictly defined, apraxia implies an absence of action, but neuropsychologists most often use it
to describe a variety of missing or inappropriate actions that cannot be clearly attributed to primary
-

motor or sensory deficits, or lack of comprehension, attention, or motivation. Thus, the term apraxia
refers to an inability to perform voluntary actions despite an adequate degree of motor strength and
control.
The adjective “voluntary” is key to understanding this disorder.

- A patient may be able to spontaneously don a jacket, for instance, but be unable to do so on
command. Therefore, family members who ask a patient to do something may perceive him or her as
being oppositional or stubborn when, in fact, he or she does not control the skills required to perform
the action.

- A typical type of task that is given if apraxia is suspected is to ask a patient to pantomime a
goaldirected action or series of actions. For example, a person may be asked, “Show me how you
would use a key to open a door.” Depending on the type of apraxia, a patient may “misperform” this
action in several ways.

- There are several subtypes of apraxia, depending on a number of behavioral variables affecting
movement and movement initiation. They are: limb-kinetic, ideomotor, conceptual, and dissociation
(formerly ideational) apraxia.

- Limb-kinetic apraxia (also ideokinetic): They appear clumsy and have poor motor control. In
attempting to show how a key would be used, limb-kinetic apraxics may make large grasping motions,
rather than fine thumb-to-forefinger movements. Because limb-kinetic apraxia is defined as a problem
in executing precise, independent, or coordinated finger movements, people with this disorder are also
likely to perform quite poorly on the finger-tapping and pegboard tests.

- The theories regarding the brain areas implicated in limb-kinetic apraxia involve the
corticospinal tract, basal ganglia, and the premotor cortex, as well as a larger frontoparietal circuit of
motor control. A progression of gross- to fine-motor control can be observed with the myelinization
of the corticospinal tract during development. As the corticospinal tract matures, children first grasp
with the entire hand before being able to execute independent and coordinated finger movements.
However, limb-kinetic apraxia goes beyond a corticospinal or basal ganglia deficit and involves
aspects of grasping and fine-motor manipulation involved with the larger frontoparietal circuit
(Leiguarda, Merello, Nouzeilles, Balej, Rivero, & Nogues, 2003).

- Ideomotor apraxia : This involves difficulties in the execution of the idea of a movement,
even though the knowledge of the action is preserved. For example, in response to a request to
pantomime use of a key, the person may mistakenly use the index finger as the key (body part as tool
error) or turn the whole arm in an unnatural fashion (movement orientation error).

- However, someone with ideomotor apraxia may be able to use a key correctly if it is put in the
hand and should recognize the correct gesture if it is performed by someone else, or if given a choice,
can match tools with correct actions.
Lesions to the left parietal lobes are commonly associated with ideomotor apraxia of either hand. It
is believed that the left parietal lobes have a special role in programming purposeful, skilled
movements, especially in those who are left hemisphere dominant for language (Meador, Loring, Lee,
Nichols, & Heilman, 1999).

- Conceptual apraxia : The knowledge of the action has been lost. For example, when asked to
gesture how to use the key, the person may perform any number of vague movements. When given a
key, he or she may try to use it as a pen or a toothbrush and, in addition, may not be able to pick out
the correct gesture if shown by someone else.

- Conceptual apraxia is not associated with damage to any one area, but is related to wider loss
of semantic knowledge of tools and actions. This problem may be seen in the early stages of dementia,
such as Alzheimer’s disease.

- Dissociation apraxia (formerly ideational apraxia) : This involves impairment in an action

sequence.
This type of apraxia can be witnessed in a multistage request such as, “Show me how you would pour
and serve tea.” Actions may be performed out of order, although the individual actions themselves are
correct. Disorders of an action sequence may be caused by executive or frontal lobe dysfunction and a
possible dissociation of action programs from language.

- In summary, the apraxias, as disorders of the how system of motor control, represent problems
with the mental representation of actions. Although we present apraxia subtypes as independent
entities, different types can occur together in the same person. The apraxias represent the most common
type of motor problems treated by neuropsychologists, because they can occur due to a wide variety
of cerebrovascular disorders (i.e., strokes), as well as with tumors or disease states, and the disability
they produce interferes with many complex actions of daily life requiring higher cortical functioning.

4. AMNESIA
Amnesia can be caused by a number of different problems and can take several forms.
-

- Anterograde amnesia is the loss of the ability to encode and learn new information after a
defined event (such as head injury, lesion, or disease onset).

-Retrograde amnesia is the loss of old memories from before an event or illness.

- For example, one case, L.S., experienced a head injury as a result of a car accident and had
moderate anterograde amnesia and mild retrograde amnesia. She had no memory of the car accident
and only vaguely remembered the paramedics. She did remember being in the hospital emergency
room. After the accident, she had difficulty learning new information (anterograde amnesia). She often
forgot her physician’s name, and when she returned to college, she found it hard to study and perform
well on history tests for which she had to recall facts and dates. Her mild retrograde amnesia is
evidenced by her memory for driving out of the grocery store parking lot, which was about five blocks
from the accident. However, she remembered nothing else of the short time preceding the accident.

-Perceptually, amnesiacs also show normal adaptive behavior when wearing visual prisms. Because
prisms distort visual input, simple acts such as reaching for an object are misdirected at first. The visual
motor system must quickly learn to “retune” the system so that it again correctly targets reaching
according to the new visual information. Interestingly, amnesiacs can do this performance learning and
adaptation despite severe declarative amnesia.

- However, amnesiacs do not show normal nondeclarative skill learning for all tasks. For
example, investigations (Gabrieli, Keane, & Corkin, 1987; Xu & Corkin, 2001) of H.M.’s performance
on a complex, nondeclarative, problem-solving task (Tower of Hanoi) did not find evidence of
consistent improvement across learning trials or mastery of the task. Further support for a
nondeclarative memory system is provided by the differential performance of patients with amnesia,
Huntington’s disease, and Parkinson’s disease on a measure of serial reaction-time skill learning
(Schacter & Curran, 2000). The patients were required to press one of four keys when illumination
occurred above a key. The patient groups were not aware that there was a repeating sequence of
illumination; yet, across learning trials, the patients with amnesia demonstrated improved performance
as evidenced in decreased key press reaction times. In contrast, patients with Huntington’s and
Parkinson’s diseases showed impaired learning.

- Memory problems constitute a major deficit for people who have sustained traumatic head
injuries and are of special interest to neuropsychologists. In fact, head injury experts grade the severity
of a head injury partly on the patient’s memory surrounding the accident. Such memory problems are
called post-traumatic amnesia (PTA). Although the patient may have residual short-term memory
impairment from the head injury, as well as other cognitive deficits, neuropsychologists have
established retrograde and anterograde amnesia as a relatively robust measure of the severity of trauma
and its associated cognitive symptoms.

-Because cortical and subcortical structures mediate memory, PTA has proved a better overall
indicator of brain damage than length and depth of coma, which may relate to isolated damage of
the brainstem. Neuropsychologists consider these types of amnesia to relate mostly to anterior
temporal lesions, an anatomic area particularly vulnerable to head injuries, because of the bony
features surrounding this area of the brain.

Childhood (Infantile) Amnesia

- This is an inability to remember events from infancy or early childhood. The early years of life
are generally regarded as critical in our development. We acquire many skills, including language,
and much knowledge in those years but for the most part do not remember the experiences through
which we acquired them.

- One reason for this failure to remember is that memory systems mature at different rates.
Personal memories of our early years may be lost because the system central to storing adult
episodic memory is not yet mature.

-Another reason that childhood memories may be lost is that the brain plays an active role in
deleting them, perhaps to make room for new memories. Katherine Akers and colleagues (2014)
find that childhood amnesia also occurs in some nonhuman animals. During the period for which
amnesia occurs in experimental mice, for example, many new neurons are being added to the
hippocampus. In precocial species, including the guinea pig, hippocampal neurogenesis is largely
complete before birth, and they do not display infantile amnesia. The investigators suggest that as
new hippocampal neurons form new connections, they participate in forming new memories, but
in doing so they disrupt neural circuits that support memories already acquired. More-permanent
memories are formed only after the acquisition of new neurons by the hippocampus slows.

Amnesias rare and common

- Fugue state : Memory loss of personal history that is sudden and usually transient. Fugue
means
-

“flight,” and one interpretation is that the person has in effect fled a former life to form a new one.
Perhaps the basis of the fugue state is the temporary suppression of medial-temporal-lobe memory
systems.

- Damage to restricted brain areas can cause amnesia that takes very curious forms. Clinical
reports describe people who become amnesic for the meaning of nouns but not verbs, and vice
versa, or amnesic for recognizing animals but are not prosopagnosic (amnesic for human faces).
Simona Siri and colleagues (2003) describe a patient with herpes simplex encephalitis who was
severely amnesic for fruits and vegetables but less so for animals and birds, suggesting a partial
dichotomy in memory between plant and animal categories.
- We all experience little everyday amnesias: we forget people’s names or faces or where we put
our keys. This kind of forgetting can increase with advancing age, in so-called senior moments. Its
onset is typically characterized by amnesias for the names of people we do not often meet and for
items we encounter in news media and in conversation. For some people, memory disorders of
aging can become incapacitating, as happens in Alzheimer’s disease, characterized by the extensive
loss of past memories and accompanied by neuronal loss that begins in the medial temporal lobe
and then extends to other brain areas.

Time dependent Retrograde Amnesia

- Traumatic brain injury (TBI) commonly produces time-dependent retrograde amnesia, with
the injury’s severity determining how far back in time the amnesia extends. For example, after a
head trauma, a transient loss of consciousness followed by a short period of confusion and
retrograde amnesia is typical.

-The retrograde extent of the amnesia (the period of personal history it covers, extending from the
present to the more-distant past) generally shrinks with the passage of time, often leaving a residual
amnesia of only a few seconds to a minute for events immediately preceding the injury.

- The duration of such posttraumatic amnesias can vary. In a classic study, in one group of
patients with severe head injuries, 10 percent had duration of less than 1 week, 30 percent had
duration of 2 to 3 weeks, and the remaining 60 percent had duration of more than 3 weeks (Whitty
and Zangwill, 1966).

- Sometimes isolated events, such as the visit of a relative or some unusual occurrence, are retained
as islands of memory during this amnesic period.
•
Three theories of amnesia

I) System Consolidation Theory

As articulated by Larry Squire and Peter Bayley (2007), system consolidation theory states that the
hippocampus consolidates new memories, a process that makes them permanent. When consolidation
is complete, the memories are stored elsewhere in the brain. That is, memories are held in the
hippocampus for a time, then gradually consolidated in a new location, the neocortex.

- Consolidation theory explains why older memories tend to survive cases of hippocampal damage—
they have been transferred elsewhere for storage— whereas more-recent memories are likely to be
lost—they still reside in the hippocampus. If damage is limited to the hippocampus, retrograde amnesia
may extend back for only a few years because only recently acquired memories remain there. As more
of the temporal lobe, a region where longer-term memories are stored, is affected, retrograde amnesia
can extend back for one to two decades or longer, depending on lesion size.

II) Multiple Trace Theory

Lynn Nadel and Morris Moscovitch (1997) propose that multiple-trace theory accounts for individual
differences in amnesias:

• In any learning event memories of many types are encoded in parallel in different brain locations.
For example, autobiographic memory depends on the hippocampus and frontal lobes; factual semantic
memory depends on temporal-lobe structures; and general semantic memory, on the remaining cortical
areas.

• Memories change throughout a person’s life as they are recalled, reevaluated, and restored.
Autobiographic events, for example, through being recalled and discussed, can also be stored as factual
memory and perhaps even as general memory (Cabeza and Moscovitch, 2013). Thus, the very process
through which memories change as they are reused places them in different brain locations. This
transformation results in memory that is recoded with use and thus changed from one type to another.

• Different kinds of memory, being stored in different locations, are differentially susceptible to
brain injury. Because of this memory organization, after brain injury usually only some aspects of
memory will be affected, and more important, older memories will be more resistant to disruption than
newer memories.
•
III) Reconsolidation Theory

- As described by Natalie Tronson and Jane Taylor (2007), reconsolidation theory proposes that
memories will rarely consist of a single trace or neural substrate. We frequently recall memories,
think about them, and discuss them with others. In storytelling or gossiping, a memory is not only
recalled but also shared and elaborated on by others.

- Each time a memory is used, it is reconsolidated: the memory reenters a labile phase and is then
restored as a new memory (Schwabe et al., 2014).

- Each use of memory is associated with a new phase of storage, resulting in many different traces
for the same event.
- Reconsolidation complicates the study of amnesia because spontaneous recall and even
investigating a subject’s memory will change the memory that is the object of investigation. For eg:
a witness’s memory for an accident consists of multiple traces, one for each time the accident is
recalled. And the difficulty in studying childhood amnesia lies in separating memories that an
individual recalls for the first time from those he or she has recalled numerous times and those that
have been contributed to by reminders from others, such as a parent (Wang and Peterson, 2014).

These three theories suggest that memory storage or memory type or frequency of use contributes to
the extent of amnesia. Because of the complexity of memory storage, we can expect wide individual
differences in the degree to which people display anterograde and retrograde amnesia after a traumatic
event.
•

Varieties of Amnesia

Unit 4: Cognitive and brain changes associated with normal aging.

COGNITIVE CHANGES ASSOCIATED WITH AGING:

• Why is there such a range of functioning in people older than 65? Does everyone lose some cognitive
functions even if they do not have degenerative brain disease? Is cognitive decline uniform, or are
certain areas more likely to decline than others? What is the trajectory of cognitive decline? Finally,
are there protective factors against cognitive decline and disease? Numerous examples exist of people
who stay active and working in their fields well past the age of retirement. Whether a scientist, a
musician, or a mechanic, these people have developed an expertise and an accumulated body of
knowledge related to their work.
• In fact, all people are likely to develop areas of expertise over their lifetimes related to work, hobbies,
or talents. This crystallized intelligence consists of stored knowledge and habitual ways of acting or
solving problems built up over a lifetime. By rehearsal, practice, and use, certain domains of
knowledge become strengthened and are more easily accessible and perhaps less subject to decay.
• Verbal scales of intelligence tests typically measure general crystallized intelligence not related to a
specific work domain. They measure factual knowledge, such as vocabulary definitions, or general
information learned in school.
• Crystallized intelligence represents an accumulation of acquired skills and general information and is
more related to formal education or diverse social experiences. Research using the Wechsler Adult
•
Intelligence Scales (WAIS-R) suggests that levels of crystallized intelligence show only slight changes
as we age (Kaufman, Reynolds, & McLean, 1989). It has also been theorized that higher levels of
crystallized intelligence or general knowledge may provide a protective factor, or “functional reserve”
(in this case, a “cognitive reserve”), against dementia that allows the brain to compensate in the
presence of declines presented by aging or disease.
• Two series of studies lend credence to the idea that a protective cognitive reserve may start early in
life. The first of these is provided by the longitudinal study of aging and AD called the Nun Study.
David Snowdon of the University of Kentucky has followed 678 Roman Catholic sisters who agreed
to regular cognitive and medical assessments and brain donation at death. Snowdon and his colleagues
seek to shed light on the factors that lead to increased longevity, as well as on the determinants of AD
and other brain disorders such as stroke. What is unique about this research is the availability of records
from young adulthood and throughout the time each nun resided in the convent. In one study, Snowdon
and colleagues (1999) examined the linguistic complexity of autobiographies written as the nuns
entered the convent between ages 18 and 32. Women who scored lower on “idea density” (that is, the
number of different ideas discussed) early in life also showed lower cognitive functioning after age
75. Also, autopsies of a small sample of nuns indicated that the women who had brain markers of AD
showed lower “idea complexity” as young women than those who did not have brain pathology.
• Snowdon’s studies used “idea density” as a proxy for intelligence, but researchers from Scotland
(Whalley, Starr, Athawes, Hunter, Pattie, & Deary, 2000) were able to actually assess the relation
between a standardized general intelligence test, administered at age 11, and signs of dementia more
than 50 years later. Children who had higher mental ability were less likely to have dementia when
they were located again at age 72. Interestingly, there was no relation between mental ability and
decline for those who had been diagnosed with an early-onset (before age 65) dementia. Early-onset
dementias, as discussed later, may be caused by disease processes that are different than late-life
cognitive decline. Both the Nun studies and the Scottish studies show a correlational link between
early intelligence and the health of the aging brain.

• One possible explanation is that a “cognitive reserve” serves as a reservoir to resist the effects of aging.
If this is so, it is not yet known whether cognitive reserve is, for example, a reflection of having more
neurons and glial cells in crucial areas or the result of wider or more efficient semantic networks. In
addition, the positive correlation between intelligence and the aging brain may be related to other
factors associated with longevity. Those with higher intelligence may be more likely to seek and follow
•
health information, have higher paying jobs, adhere to a better diet, and have better access to health
care.
• Even people who show little signs of cognitive decline, however, are likely to notice changes in fluid
intelligence. Areas of fluid intelligence involve novel reasoning and the efficiency of solving new
problems or responding to abstract ideas. Fluid intelligence has also been conceptualized as a measure
of adaptability. Fluid intelligence is most directly related to the influences of changing biological
factors and is relatively unaffected by higher levels of experience or education. The most reliable
declines in cognition show up in three areas of intellectual activity, all of which are considered fluid
markers of intelligence: (1) processing speed, (2) abstract and complex new problem solving, and (3)
memory and new learning.
• Tests of fluid intelligence (for example, Wechsler’s Digit Symbol and Block Design) generally require
both novel processing and the ability to complete a task quickly. Studies of aging suggest that
performance on tests of this type declines across the life span (Salthouse, 1991). Behavioral slowing
also occurs and may partly contribute to poorer performance on a number of tests of fluid intelligence
where speed is a factor, but age-related decline is still observed on novel problem-solving tasks even
in the absence of speed demands.
• Studies of aging suggest that older people are more likely to have more difficulties in many aspects of
memory. Early research in aging and memory suggested that the main problem for older people was
information retrieval. Poorer performance occurs with free recall, compared with recognition, with
less contextualized information, and when more effort is involved. However, new learning may also
be difficult because of problems in encoding, particularly with intentional encoding. In fact, some of
the retrieval issues relating to poorly remembered contextual information may be due to poor encoding
of context at the outset. Once information is encoded, however, healthy older adults seem to show
similar semantic storage in long-term memory as younger adults.
• Semantic storage can be conceptualized as drawing more heavily on crystallized intelligence and
knowledge structures (Bäckman, Small, Wahlin, & Larsson, 2000). There also appears to be little
effect of aging on procedural and implicit memory tasks, although these may be performed more
slowly. It has been predicted that older adults would perform more poorly on prospective memory
tasks because of a high degree of self-initiation required in remembering to do something in the future
(for example, McDaniel & Einstein, 2000).
•
• However, because prospective memory requires both remembering “what” is to be done and “when,”
it appears that older people have more trouble with the “what” or content that may have more to do
with basic encoding, storage, and retrieval mechanisms in retrospective memory (for review, see
Henry, MacLeod, Phillips, & Crawford, 2004). Although older people may perform more poorly on
laboratory prospective memory tasks, they do much better on real-life tasks, such as keeping
appointments or remembering to post mail or return phone calls; tasks for which motivation may be
different or external reminder aids may come into play (for review, see Henry et al., 2004).
• A number of researchers suggest that working memory (WM) capacity declines with age. Interestingly,
short term memory, or the ability to recall strings of digits, does not appear to decline with age.
However, this is a more passive task than WM, where information must not only be held but also
manipulated and processed in a more complex manner.
• How stable is cognitive functioning among those 75, 85, or older? Does the pattern look the same as
one continues to get older? Do initial losses of function stabilize, is there a gradual decline, or is there
an acceleration of loss in some areas of functioning? In a review of studies of longitudinal aging, it
appears that the pattern of preserved crystallized intelligence over fluid intelligence does not hold in
those adults older than 75 (Bäckman et al., 2000), and all intellectual abilities show a decline in group
studies.
• However, a series of interesting studies conducted in Sweden document the neuropsychological
performance of the oldest segment of the population. In one study, researchers gave
neuropsychological tests twice, 2 years apart, to more than 300 people between the ages of 84 and 90
(Johansson, 1991). This study of the oldest old (84–90 years old) found surprising stability in
neuropsychological functioning between the first and second test sessions. Researchers expected that
functioning of people at this advanced age would decline over 2 years. However, two thirds of the
sample (66%) remained at the same cognitive level, whereas 31% declined (Johansson, Zarit, & Berg,
1992).
• Almost half (42%) remained in the normal range of functioning during the 2-year time period.
This finding was surprising not only in that a large portion of the sample showed stability of
cognitive function over time but also that a significant portion of quite elderly adults still had
“normal” cognitive function.
• Johansson and his colleagues (Johansson, 1991) suggest that cognitive changes were more
related to terminal decline, or proximity to death, than to chronologic age. Among other
•
neuropsychological tests, these examiners administered the digit span task, at regular intervals,
to normally aging Swedes older than 70.
• This requires repeating increasingly longer series of digits either in sequential or reverse order,
respectively, until the testee misses them. For the 70- to 88-year-olds studied over time,
Johansson examined two groups: those who died before age 85 and those still living. Those
alive at age 85 showed a consistent performance as they aged; those who died before age 85
started showing a drop in backward digit span by age 75 and marked declines in both forward
and backward span lengths by age 79.
• What then is the secret of people who are active and productive well into their later years? In
recent years, much focus has been on what can be termed the “use it or lose it” hypothesis.
This idea suggests that by keeping mentally active, or by increasing mental exercise, older
people may be able to stave off mental decline and diseases of aging. The question is, does
mental exercise, such as doing crossword puzzles, starting a new hobby, or memory training
help to slow or reverse the affects of aging?
• This hypothesis has also been called the differential-preservation hypothesis (Salthouse,
Babcock, Skovronek, Mitchell, & Palmon, 1990) because it is assumed that the large age
differences in cognitive functioning seen in the oldest adults are due to differences in their
current levels of mental activity and mental exercise. This is in contrast with the preserved-
differentiation hypothesis (Salthouse et al., 1990) that is more in line with the idea of
“cognitive reserve” discussed earlier in this section. In other words, it may be that the range of
cognitive differences found among older people are because those who showed higher
cognitive ability to begin with continue to show this pattern as they age.
In a recent review and commentary on this issue, Salthouse (2006) suggests that the research
on “mental exercise” or training as a strategy has not yet demonstrated convincing results. For
example, research focused on training people on various cognitive tasks, although showing some
immediate benefits in targeted task performance, has not shown to be generalizable to other tasks,
often in the same cognitive domain.
Also, the effects of training are not convincingly sustained over time compared with those who
were not trained. Therefore, although there is much optimism in the popular press about our
potential ability to stave off the general effects of aging through mental exercise, this idea does not
yet appear to be supported. Although the mechanisms are not yet known, people who continue to
•
be active well into their 70s, 80s, and 90s may be the best at resisting both declines in fluid and
crystallized intelligence.
• They may have started out with a higher level of crystallized intelligence, and thus are provided
with a certain degree of cognitive reserve. They may have learned a great deal in their life,
which also provides them with strong crystallized semantic networks. Although aspects of fluid
intelligence such as speed and flexibility of thought may decline, many older people do
maintain an active and independent lifestyle well into their later years.

BRAIN CHANGES ASSOCIATED WITH AGING:

• Variation in functional abilities with aging is a clue that the brain may not decline in a uniform manner.
But how does it age, and when is change noticeable? By reviewing both global (structural and
neuronal) and regional brain changes affected by aging, as well as considering the trajectory of decline
and factors that impact brain aging, a picture emerges of how the brain changes as people grow older.
The aging brain undergoes visually apparent gross structural changes such as diminution in size and
weight, flattening of the cortical surface, and expansion of the cerebral ventricles. The loss of weight
and volume occurs in a general linear trajectory (for review, see Raz, 2000).
• One of the first recognizable global indexes of brain health or shrinkage is widening of the ventricles.
If the brain loses volume in any area, the ventricles reflect this. However, this gross marker does not
necessarily imply that the brain loses volume equally across all areas.
• Concomitant changes occur at the neuroanatomic and biochemical levels. Neurons undergo significant
structural changes with aging. Aging cells may shrink and die, lose some of their dendritic processes,
and develop a yellowish brown pigment that accumulates in cells of the cortex and cerebellum and
may have to do with “wear and tear” (Bourne, 1973; Kemper, 1994).
• Observations of cortical thinning may have led to one of the myths of human neurobiology, namely,
that throughout adulthood people lose a great number of neurons from their brains each day. Better
measurement methods indicate that this is exaggerated, and that much cortical thinning may be due to
neuronal shrinkage rather than loss (for review, see Haug, 1985).
Although some markers of neuronal abnormalities such as neurofibrillary tangles and senile plaques
(see Figures 14.6 and 14.7) are hallmarks of AD and other dementias, they also occur in older people
without frank evidence of cognitive dysfunction.
•
• Images of aging brains often show white matter abnormalities indicating attenuation of myelin around
the axons of neurons. This observation has led a number of researchers to question whether white
matter (that is, myelinated axons) or gray matter (that is, cell bodies) may succumb more quickly to
the aging process.
• Cerebrovascular disease and hypertension, both more common in older adults, are associated with
white matter abnormalities (for example, Strassburger et al., 1997), but these comorbid problems of
aging do not appear to fully explain white matter aging. In an analysis of studies across the life span,
gray matter suffers a linear decline from infancy through old age, whereas white matter shows an
inverted U-shaped function with increasing white matter into young to middle adulthood, followed by
a plateau and then a decline into old age (for review, see Raz, 2005).
• The brain shows differential changes with aging. Although some brain areas appear more
vulnerable to the effects of aging, there are islands of relative preservation. The hippocampus,
the frontal lobes, and specific association areas of the temporal and parietal lobes are more
vulnerable, whereas the occipital and somatosensory cortices are relatively preserved. The
frontal cortex is one of the cortical areas most affected by aging. The most likely set of age-
related neuronal changes specifically affects the prefrontal cortex (Esiri, 1994).
• Neuronal loss in this area may account for some of the fluid intelligence changes in cognitive functions
occurring in older people. Because cognitive functioning varies widely among older people, it is also
reasonable to assume that there is a range of individual variability in physical brain changes.
• When assessing the degree of cortical atrophy caused by advancing age, gross inspection of the brain
demonstrates wide variation (Figure 14.2). In the Swedish study (Johansson, 1991), 85% of elderly
adults’ brains appeared to have little to no evidence of cerebral atrophy. However, all did show some
neuropathologic markers usually associated with dementia, including signs of ischemia (that is,
insufficient blood supply), neurofibrillary tangles, and senile plaque formations.
• In individuals older than 85, gray matter atrophy is often apparent on computed transaxial tomography
(CT) in both demented and nondemented people. White matter attenuation (thinning of the white
matter) relates to cognitive changes associated with fluid intelligence, such as slowed speed of
behavior, poorer spatial ability, poorer arithmetic, and memory recognition skills (Johansson, 1991).
• Genetic and environmental factors also play a role in an individual’s vulnerability to brain aging. A
variety of genetic factors have been implicated in the dementias discussed in this and the next chapter.
•
Some of these genetic factors may also prove to accelerate the aging process in people who do not
develop a full-blown dementia. For example, a specific allele of apolipoprotein E (that is, ApoE4) has
been implicated in some forms of AD.
• However, ApoE4 may also be implicated in general problems of white matter maintenance through its
action on the cholesterol system of the fatty oligodendrocytes that make up the myelin sheath and
through a disruption in the maintenance of intracellular calcium balance (Masliah, Mallory, Veinbergs,
Miller, & Samuel, 1996; Raz, 2000).
• It is generally accepted that prolonged stress has negative effects on health.
• However, studies of stress and aging suggest that stress may age both immune and brain cells. Immune
cells contain chromosomes with end caps termed telomeres. Telomeres shorten as cells reproduce and
are a measure of the life of the cell. In a study of women who were continually under high stress levels
because of caring for chronically ill children, it was found that their telomeres had undergone the
equivalent of 10 more years of aging as compared with women who were living less stressful lives
(Epel et al., 2004). Stress also appears to affect certain brain areas.
• For example, people who have high basal cortisol levels (a biochemical marker of stress) show reduced
hippocampal volumes over time (Lupien et al., 1998). Whereas stress may negatively impact the brain
and cognition, aerobic activity appears to enhance it. Older people who engage in regular aerobic
activity perform better than sedentary people on a wide range of cognitive tasks (for review, see
Colcombe & Kramer, 2003).
• In direct measures of brain density, it has also been reported that exercising older adults showed
reduced loss of gray matter in frontal, temporal, and parietal areas, as well as less reduction in white
matter tracts in both anterior and posterior brain areas (Colcombe et al., 2003).
 Unit 5-Neuropsychological defects associated with stroke, brain tumours and head injuries

BRAIN TUMOR.

Neoplasm- New growth

Neoplasm is identified as a mass of new cells with no useful purpose and these can be benign or

malignant. Benign

Grows from the covering of the brain i.e., the meninges and the tumors arising from it is called the
meningioma’s. This type of tumors grows slowly and attain large size before they cause symptoms/
sometimes they are symptomless. Can be successfully removed since they don’t invade brain
tissues/substance.

Malignant.

Arise from the glial/supporting cells termed primary neoplasm (were the cancer began originally)
one of the common type is Glioma (40% of adult tumor is this type). Secondary neoplasm is a new
primary cancer/a spread of the original one from another region.

Space occupying lesions (SOC).

Conditions that are not neoplastic but very similar in signs and symptoms

Ex: Puss in brain

But the true nature may not be identified until an exploratory operation. These SOC will produce a
set of general symptoms but each particular case will be having specific features depending on the
interruption of connection between different brain parts.

STROKE/CEREBRAL HEMORRHAGE.

There is a large number of causes for stroke but the most seen 3 conditions include Hypertensive
intracerebral hemorrhage, Ruptured aneurysms, Ruptured arteriovenous malformations.

Hypertensive intracerebral hemorrhage.

 Most common form

 Bursting out of blood into the substance areas of the brain and the bleeding destroys the brain tissues
and if it is continued it causes pressure effects in neighboring tissues.
 Less pressure may primarily disrupt the function of adjacent areas or nearby tissues without
destroying it so for the full development of it takes time because it depends on the rate of bleeding
and final cessation.

Ruptured aneurysm.

 Arterial circle/circle of Willi- Formed of thin wailed protrusions

 Developmental defect in Arterial wall and they are prone to rupture
 Mostly these are seen in anterior communicating region and middle cerebral bifurcation and there
can be multiple aneurysms too.
 During the rupture the blood may be spurted into the subarachnoid space or into the adjacent brain
areas/ substance causing intracerebral hematoma.

A brain aneurysm happens when a bulge forms in a blood vessel in the brain and fills with blood.
Aneurysms often produce no symptoms unless they burst open or leak blood. A ruptured aneurysm
causes severe headache and can lead to a fatal stroke.

A brain aneurysm is a bulge in a weak area of a blood vessel in or around your brain. The constant
pressure of blood flow pushes the weakened section outward, creating a blister-like bump.

When blood rushes into this bulge, the aneurysm stretches even further. It’s similar to how a balloon
gets thinner and is more likely to pop as it fills with air. If the aneurysm leaks or ruptures (bursts
open), it causes bleeding in your brain.

Sometimes it causes a hemorrhagic stroke, bleeding in or around the brain that can lead to brain
damage and be fatal. These aneurysms are also called cerebral aneurysms. Cerebral means in the
brain.

Ruptured arteriovenous malformations (AVM)

 Small localized abnormalities of a few millimetres long to a large mass of cells.

 The blood vessels forming the mass of interposed between the feeding arteries and the
draining veins are pathologically thin-walled and liable to rupture
 Rupture of large malformations can cause subarachnoid bleeding and this AVM has a
tendency for recurrent rupture like the Saccular aneurysms.
 A brain arteriovenous malformation (AVM) is a tangle of abnormal blood vessels connecting arteries
and veins in the brain.

The arteries are responsible for taking oxygen-rich blood from the heart to the brain. Veins carry the
oxygen-depleted blood back to the lungs and heart. A brain AVM disrupts this vital process.
An arteriovenous malformation can develop anywhere in your body but occurs most often in the
brain or spine. Even so, brain AVMs are rare and affect less than 1 percent of the population.

Unit 6: Neuropsychological defects associated with cortical and subcortical dementias

DEMENTIA

• The term dementia in its broadest sense refers to a group of conditions and diseases that share some
similar neuropsychological and behavioural symptoms, although the underlying causes may vary
widely.
• The prime identifying feature is a decline in multiple areas of cognitive functioning, including
memory. The dementia syndrome is a cluster of behavioural symptoms that may or may not point to a
disease, but dementia is not a disease entity in and of itself.
• The various subcategories of dementia usually relate to the suspected disease, cause, or primary site
of damage (for example, cortical versus subcortical). Researchers have found well over 50 causes of
dementia. Among the most well-known are the degenerative dementias caused by a progressive and
unrelenting disease process such as AD or Parkinson’s disease.
• Neurologists traditionally have categorized these disease processes as cortical, subcortical, or mixed,
depending on the degree to which they affect grey or white matter areas of the brain.
• Vascular, infectious, and toxic conditions, as well as a variety of other brain conditions, may also result
in dementia.
• Some of these conditions are progressive, whereas others, such as the dementia resulting from herpes
encephalitis, may be static, rarely worsening over time. Although most dementing conditions
encountered by neuropsychologists represent persistent or progressive states, or both, researchers have
also documented “reversible” or temporary dementias.
• Reduced metabolic efficiency accompanies aging, making older adults especially susceptible to
conditions and substances that they might have tolerated when younger.
• For example, symptoms of dementia can stem from adverse reactions to medications (such as sedative
hypnotics and anticholinergic drugs), nutritional disorders (such as thiamine deficiency and pernicious
 anemia), metabolic disorders (hypoglycemia, hypercalcemia, kidney failure), psychiatric disorders
(severe mood disorders, psychosis), and other conditions such as anesthesia or surgery. However,
when these conditions are treated, the dementia is usually reversible and the patient returns to baseline.

Diagnostic Criteria for Dementia

a. The first is that dementia results in a loss of cognitive or intellectual function. This feature implies a
decline that is acquired and unusual. It is acquired because people born with impaired intellectual
function, having developmental disorders such as mental retardation, do not have dementia simply by
virtue of poor intellect, although they too can experience development of dementia. The loss of
cognitive or intellectual functioning must also be unusual or outside of the realm of what would be
expected with normal aging.
b. Dementia involves multiple areas of cognitive impairment. The abilities impaired in dementia may
represent all cognitive functions or may present different patterns of neuropsychological disability. It
is not uncommon to see impairment in abstract thinking and problem solving, impaired judgment, and
other problems of higher cortical functioning.
Subtypes of Dementia
 Cortical versus Subcortical
• Cortical dementias primarily affect, or start out by affecting the cerebral cortex, or grey matter.
AD is typically included within this category. AD typically causes significant cortical neuronal
loss and atrophy, but also specifically attacks the hippocampus, a subcortical limbic system
structure
• Subcortical dementias, the disease state predominantly affects the white matter, or neuronal
connections between cortical areas, and grey matter structures below the cortex. The term
subcortical was first used to describe the neuropathology and accompanying pattern of
cognitive deficits associated with progressive supranuclear palsy.
• Diagnosticians usually identify Parkinson’s disease by the subcortical structure that it targets,
the substantia nigra, although evidence suggests that it also affects some higher cortical
functions such as executive functioning.
 Static versus Progressive
All dementias that result from a disease process are progressive. Diseases such as AD, Pick’s,
Huntington’s, or Creutzfeldt–Jakob inevitably follow a continuous cognitive and behavioural
decline.
 Other conditions, however, may cause a static or steady-state cognitive disorder.
 A neurotoxic substance (such as lead or alcohol) or infection (such as herpes
encephalitis) continues to cause brain damage as long as it is present. But when the
condition is arrested, the resultant dementia usually plateaus.
 Both static and progressive dementias can begin with a sudden change of functioning,
over days or weeks, or a more insidious or gradual onset, over the course of months
or years.
 The progression, as in the case of AD, is gradual. However, there may be long periods
during which the decline plateaus.
 Vascular dementias often produce a stepwise progression, as multiple infarcts
(multiinfarct dementia) or strokes occur at different times.
 Only repeated neuropsychological testing and keen observation
by the
neuropsychologist, patient, or family can demonstrate the progression of the dementia.

• Reversible versus Irreversible

 Delirium does not signal dementia, but rather is a transient cognitive problem
associated with an acute confusional state.
 Individuals with delirium have poor attention, disorganized thinking, perceptual
disruption, disorientation, memory impairment, and an altered state of consciousness.
 Because delirium and dementia share memory impairment and disorientation, they
can be easily confused.
 With delirium, the symptoms develop over a period of days or hours and are caused
by specific organic problems such as overmedication or an acute or worsening
medical condition.

CORTICAL DEMENTIAS
Alzheimer’s Disease
 Alzheimer’s disease (AD), named after its discoverer, Alois Alzheimer, is a progressive cortical
dementia that is irreversible and thus results in an inevitable decline.
 It is the most prevalent of the dementias, representing the eighth leading cause of death overall for
people older than 65 and more than 50% of diagnosed dementia cases. The number of new cases of
 AD increases with age from 1% of the population aged 65 to 75 years to 6% to 8% of adults older
than 85.
 The rate of survival varies widely between 2 and 20 years with a median survival rate of between 3
and 4 years after diagnosis.
 The causative factor of AD remains unknown. AD is linked to increased age, which has led some to
speculate that it is a disease of “accelerated aging”.
 AD would be inevitable. Over a lifetime, women are about twice as likely to experience
development of dementia or AD as men, but this may be partly accounted for by that women have a
longer life expectancy.
 People with more education appear less likely to experience development of AD, but again, this is
probably a marker of larger cognitive reserves acting as a buffer between neuropathology and
disease manifestation.
 People born with Down’s syndrome, or trisomy 21 (named because the disorder results from an
abnormality on chromosome 21), inevitably develop a dementia, usually by age 40.
 AD is difficult to diagnosis because there are other dementias that have may have similar symptoms,
especially in the later stages of the disease. Definitive diagnosis of AD requires the behavioural
presence of dementia and the identification of neuropathologic markers of AD. No single medical
test, imaging procedure, or behavioural test can positively identify AD (for example, Mayeux, 2003),
short of a brain biopsy showing the characteristic neurofibrillary tangles and neuritic plaques, which
are most predominant in the hippocampus and cortical association areas.
 The clinical diagnosis of AD depends largely on evidence related to behavioural and
neuropsychological profiles and on ruling out all other identifiable causes of dementia.

Major Brain Structures Affected by Alzheimer’s Disease

 The disease targets specific regions of the brain. Second, disease-targeted structures sustain
neuronal loss and atrophy.
 Although AD is a “cortical dementia,” because major areas of the cerebral cortex show brain
shrinkage. It greatly affects major subcortical limbic system structures such as the hippocampus and
amygdala.
 Most pathologic changes occur in the cortical temporoparietal association areas and the subcortical
limbic cortexes. Specifically, the disease destroys the major pathways to and from the hippocampus,
cutting off direct connections to association cortices.
 The most marked atrophy is in the frontal, temporal, and parietal areas. The gyri are thinned, and the
sulci are noticeably widened. Researchers estimate that in AD about half of the large neurons
deteriorate resulting in a loss of volume.
Specifically, these neurons lose dendritic arborization, or branching. The ventricles also enlarge,
because of cortical.
 Although dementia severity increases with increased cell death, longitudinal comparisons of global
cerebral atrophy with dementia severity do not reliably indicate dementia.
 The affected structures correspond to areas of higher cognitive functioning and memory, leaving
relatively untouched more basic sensory and motor abilities.

Neuropathology

 The two neuropathologic findings that Alzheimer identified, still considered the primary markers of
the disease, are neurofibrillary tangles and neuritic, or senile, plaques.
 Tangles consist of proteins, termed tau proteins, that are believed to accumulate as a result of
abnormal phosphorylation. The excessive collection creates tangles that are dispersed throughout
the brain but disproportionately in the areas just listed, including the temporoparietal areas and the
hippocampal complex.
 The specificity of structural deterioration in AD extends to the cellular layers of the cortex.
 Plaques are round aggregates of “cellular trash” that have a particular affinity for the regions where
the majority of synapses lie.
 The neuritic plaques found in AD contain an amino acid peptide protein core termed beta-amyloid.
These neuritic plaques are also called amyloid plaques.
 The most consistent evidence of a neurotransmitter with a direct effect on memory processes in AD
is acetylcholine (ACh).

Clinical Presentation

Memory, speech, Visuospatial Difficulties.

AD globally and profoundly impairs memory. New declarative learning problems at all levels
(encoding, storage, and retrieval) and retention over time are usually noticed first. One of the first and
most prominent symptoms of AD is a deficit in new declarative learning (sometimes termed
anterograde amnesia to differentiate it from retrograde amnesia [deficit in remote recall]).
 People with AD show impairment in encoding, consolidation, and retrieval. Retrieval cues will not aid
AD patients’ recall of information. Neuropsychologists noticed that patients with AD do not organize
new information semantically as they are attempting to learn it. This leads to ‘semantic clustering’.
Patients with AD appear to lose conceptual knowledge.

• AD patients show an anomic aphasia, characterized chiefly by word-finding and naming difficulties.
As the dementia progresses, language problems become more profound.
• Visuospatial difficulties are also seen in patients with AD. They have difficulties in understanding
orientations and spaces. People with AD do poorly on a number of tasks that require reasoning and
problem solving.
• Crystallized intelligence which refers to accumulated knowledge over the life span seems to be intact
in patients with AD. They also show bradyphrenia, or extremely slow information processing speed
characteristic of patients with subcortical dementias.
• Many patients with AD appear to lose metacognitive awareness, or the inability to self-monitor their
own behaviour and performance. Suspiciousness of others and frank paranoid delusions can manifest
memory dysfunction.

Treatment (Refer to page 420, Principles of Neuropsychology- Chapter 14)

SUBCORTICAL DEMENTIAS

 Parkinson’s Disease
 Huntington’s Disease
 Creutzfeldt–Jakob Disease

Subcortical dementias are so named because, although these conditions often affect cortical areas and
functioning, the structures that are prominently damaged are subcortical.

Parkinson’s Disease (PD)

 Parkinson’s disease (PD) is a slowly progressive disease of the dopaminergic system that largely
affects older adults.
Later stages of the disease are associated with dementia. PD, or idiopathic parkinsonism as it is also
known, is the most common manifestation of parkinsonism.
 Parkinsonism, like dementia, does not refer to a particular disease, but rather to a behavioural
syndrome marked by the motor symptoms of tremor, rigidity, and slowness of movement.
 This cluster of motor symptoms may be caused by PD but also by drugs, encephalitis, toxins such as
carbon monoxide and manganese, and injury.
 PD appears more common in men than women, although no differences in risk factors have been
identified.
 Younger patients with PD are likely to function well, but dementia is more likely with increased age
and disease severity.

Neuropathology

 PD is marked by a degeneration of dopaminergic cells and pigmentation in the substantia nigra

(black substance).
 It is also characterized by Lewy bodies, which are small, tightly packed granular structures with
ringlike filaments found within dying cells.

Clinical Presentation

 The diagnosis has usually been well established from the characteristic resting tremor and allied
motor symptoms.
 Physicians may refer patients to either a psychotherapist or a neuropsychologist to aid in diagnosis
before the “classic” symptoms appear. The patient may first sense vague aches and pains and wonder
whether arthritis is developing. A general feeling of tiredness or malaise may come first, which could
easily be attributed to overwork or “burnout.” Other PD patients may first report feeling irritable or
depressed. the person notices weakness in an arm or leg, including problems in writing, holding a pen,
or typing. Voice quality becomes softer and more monotone, and facial expression appears flat to
others.

Motor Symptoms

The motor symptoms of PD generally fall into groups of positive and negative symptoms. Positive
symptoms indicate an excess of motor behaviour, or abnormal motor reactivity, whereas negative
symptoms indicate a diminution or loss of motor functioning.

 The positive symptoms of PD are perhaps more noticeable, and most people recognize them as the
hallmarks of the disease. Chief among these are a resting tremor and rigidity.
 Resting tremor, as opposed to a cerebellar intention tremor, is often characterized as “pill rolling.”
This rhythmic shaking, often first occurring in one hand or the other, looks as if the person might be
rubbing or rolling a coin or pill between thumb and forefinger.
 The tremor stops or lessens with voluntary movements such as reaching, swinging the arm, grasping,
or manipulating objects. When the person is sleeping, the tremor usually disappears.
 The degree of tremor at any one time is partly due to the voluntary–involuntary nature of the
movement, the level of alertness, and the level of stress.
 Rigidity, the other major positive symptom, occurs as a tightening of muscles and joints. Muscles
may appear tense and feel contracted to touch, even when the person is relaxing.

 This increasing rigidity may result in the characteristic stooped or hunched posture of PD.
 In addition to a more rigid posture, poor balance and the inability to adjust posture may be evident.
 The inability to catch oneself quickly, or impaired righting reflex, may appear if the person is pushed
or missteps.

Visuospatial Difficulties

Many studies have found that patients with PD perform poorly on spatial tasks that have a motor
component. Those with more left-sided motor impairment may show more right hemisphere damage
(visuospatial deficits). Some studies include patients in more advanced stages of the disease.

• Some patients with PD may have a comorbid dementia. patients with PD may report having
difficulty orienting themselves in space. Spatial abilities require the person to visualize the relative
position of objects in three-dimensional space, and to make a motor response to orient himself or
herself or other objects in that space.
• Disease could theoretically disrupt this network in the parietal lobes or anywhere along the
visuomotor system.
• Some investigators have suggested that the basal ganglia play a role in the visuomotor aspects of
visuospatial problems in patients with PD.
• Although it is not clear whether all patients with PD experience visuospatial dysfunction, a sizable
proportion does. Certain subgroups of patients, or those in more advanced stages of the disease, for
example, may show the most difficulty.

Difficulties in executive functioning

• Among executive dysfunctions reported in the literature are difficulties with changing mental sets,
maintaining mental sets, and temporal structuring. The inability to switch mental set in response to
environmental demands, or perseveration, shows most clearly on neuropsychological testing through
measures that require strategy shifts to solve problems (such as the Wisconsin Card Sorting Test) or
an alternating response between two different types of stimuli (such as the Trail Making Test B or
the Stroop Test).
• The perseverative problem in maintaining set occurs after the patient tries a new or different
strategy.

It is a tendency to revert back to a previous strategy after switching “mental set.”
• Temporal ordering is an executive problem that interacts with memory.

Speech Difficulties

• PD typically does not produce classical aphasia.

• Patients with PD lose voice amplitude and vocal emotional expression (dysphonia), which results in
monotonous voice.
• Other speech irregularities may include segmented accelerated bursts of speech (tachyphemia) and
compulsive word or phrase repetition (palilalia).
Memory

Compared with AD, memory functioning is relatively spared in PD, even in patients with PD with
dementia (Sagar et al., 1988).

• Digit repetition and block-tapping repetition are usually preserve. Patients with PD may show
difficulty in motor skill acquisition.
• Strategic memory processes for organization and retrieval of declarative information are defective.
• Nondeclarative learning, which relies on intact motor or executive functioning, is often deficient.
• The ability to learn new motor skills declines as the disease progresses.
• Patients with PD without dementia can passively register declarative information in short- and
longterm memory. However, many have trouble using this information effectively. This includes
effectively organizing information to be recalled, maintaining a consistent mental set when trying to
learn or retrieve information, and time tagging or knowing not only that something has occurred but
“knowing when” it happened.

Mood and Emotional Difficulties

A large proportion of patients with PD suffer from depression. These patients may have difficulty in

showing an angry face or a surprised face but may be able to recognize emotional expression.

Treatment for PD (Refer 431, chapter 15, Principles of Neuropsychology) .

Huntington’s Disease (HD)

 HD is a progressive subcortical dementia. This rare disease, which affects about 5 to 10 in 100,000
people, is linked to the gene ITI5 on chromosome 4 and is passed on by one parent in an autosomal
dominant inheritance pattern.
 George Huntington, in his article described the disorder most clearly and most completely. He
emphasized the emotional and psychological aspects of the disease, describing “the tendency to
insanity, and sometimes that form of insanity that leads to suicide.”

Neuropathology

 The caudate nucleus is one of the structures that comprise the striatum, together with the globus
pallidus and putamen. The striatum is part of the basal ganglia, which is responsible for modulating
motor activity.
 There is loss of cell mass in the caudate and a widening of the ventricles. By the end stages of the
disease, the frontal lobes may also shrink by 20% to 30%.

Clinical Presentation

Many of the cognitive difficulties of patients with HD likely stem from a breakdown in premotor
frontal lobe functioning and the connectivity of the caudate-frontal system.

• Early in the disease process, patients with HD show characteristic frontal signs of rigidity,
perseveration and difficulty switching mental set-in daily life. Some dysfunctions stem from the
impacts that poor executive organizational abilities and attention/concentration problems have on
cognitive functioning. For example, the memory difficulties of patients with HD appear largely
attributable to poor executive functioning.
• Patients with HD appear to have difficulty orienting themselves in space, which may be a parietal
dysfunction.
• The emotional difficulties experienced by many patients with HD likely result from prefrontal and
limbic system interactions.
• Patients with HD exhibit a disproportionately high degree of affective disturbance, in the form of
depression and manic depression. The suicide rate of 6% in HD is greater than in other degenerative
disorders.
• Depression is the most common affective disturbances. Other difficulties include anxiety, apathy,
irritability, impulsivity, aggression, sexual disturbance, schizophreniform thought disorder, and
psychosis involving hallucinations and delusions.
• The motor difficulties of HD are characterized by chorea: twisting, writhing, undulating, grimacing
movements of the face and body. patients with HD walk with a wide-based gait.
• Their speech is dysarthric, becoming increasingly erratic in its rate of production and staccato with
intermittent pauses.
• They become clumsy and uncoordinated, unable to do fine-grained work.

Creutzfeldt–Jakob Disease (CJD)

 CJD is a quickly progressive subcortical dementia estimated to affect only 1 person in 1 million
people per year. This is extremely rare even in comparison with HD, which affects 5 to 10 per
100,000 people.
 Most cases have been in their 50s or 60s. For the most part, researchers have hypothesized that CJD
spontaneously arises as a random mutation.
 CJD is a compelling disease, unlike the other dementias we have considered, because of both its
speed of progression and mode of transmission.
 With a malignantly cascading decline over 3 to 4 months, it is the most quickly progressing
dementia.
 Scientists have long known that humans can transmit this disease via transplants of affected neural
tissue, cornea transplants, or contamination via medical procedures, but it is now also becoming
clear that CJD and its variants can cross species through the consumption of tainted meat containing
neural tissue.
 Extensive spongelike holes appear in the brains of its victims, giving it the fitting name “spongiform
encephalopathy” (SE).
 Researchers have identified SEs in species from minks to sheep (scrapie) to cows (bovine
spongiform encephalopathy [BSE] or “mad cow disease”) to humans (CJD and kuru).

Neuropathology

 The cause of CJD has eluded scientists until recently because it is a transmissible or infectious agent
with none of the usual symptoms of acute infection. In fact, scientists first thought that kuru could be
genetic, because it occurred primarily among the women and children of the Fore people.
 No one knows from where this infectious agent originally arose. CJD, like kuru, attacks the
cerebellum, but it also damages the cerebrum.

Clinical Presentation

The motor symptoms are those expected of cerebellar and subcortical dysfunction.

• Movements become uncoordinated, walking resembles a drunken stagger, and speech is slurred and
inarticulate.
• Involuntary tremors and choreiform grimaces emerge, and finally victims cannot swallow and thus
may die of starvation.
• Visual function alters, eventually leading to blindness in some people.
• Emotionally related complaints of mood disorders such as anxiety, depression or hypomania,
fatigue, difficulty sleeping, and attention/concentration problems.
• The dementia of CJD and its variants has a rapid progression, typically less than a year and usually
within 3 to 4 months. Kuru has a similar progression.
Neuropsychological testing of patients with CJD is rarely done, not only because of the rarity of the
disease, but also because of its circumstances. By the time the disorder is identified, patients are
untestable. Unfortunately, at this time, there are no treatments and no cure.

MODULE 3 – FUNCTIONAL UNITS OF FRONTAL LOBE

Unit 1: Basic Anatomy of frontal lobe

➢ Frontal lobe is the most recently developed parts of the brain.

➢ In humans, frontal lobe makes upto 1/3rd of the mass of the cerebral hemisphere.

➢ It lies anterior to the central sulcus and is divided into four subdivisions.

➢ Both prefrontal area and basomedial portion of the lobes together is called the prefrontal region.

➢ Frontal eye field is a specialized area situated in the middle zone or Steve dorsolateral surface
which is parts of areas 9, 8 and 6.
➢ Stimulations particularly in area 8 causes eye movements most often to contralateral. The area is
thought to a mediator of voluntary and involuntary eye movement and is situated to receive
information from the prefrontal cortex for relay to motor system.

➢ Frontal lobe works for cognitive functions higher motor movement personality and control.

➢ The frontal region has well developed systems of efferent nerve cells leading from the cortex to
the lower brain centers and peripheral parts of the nervous system. the efferent projections from the
frontal areas pass to the ventral and dorsomedial nuclei of the thalamus as well as to numerous other
structures.

➢ Afferent fibers reach the frontal cortex over the thalamo - frontal radiation.

➢ Luria perceived the organization of frontal regions in a hierarchical arrangement.

▪ Motor area - which occupies the precentral gyrus

▪ Premotor area - which lies anterior to the motor area including Brodmann area 6 and part of area 8.

▪ Prefrontal area - which includes area 9,10,45 and 46

▪ Basomedial portion of the lobes

Motor Area

➢ Motor area projects to spinal cord spinal motor neurons to control limb, hand, foot and digital
movements and to appropriate cranial nerve motor neurons to control facial movement.

Premotor Area

➢ Premotor media influence movements directly into the corticospinal projections or indirectly
through projections to the motor cortex.

➢ They receive information from the posterior parietal area.

➢ These areas are concerned with execution of limb movement.

Prefrontal Area

➢ Prefrontal area receives projections from the dorsomedial nucleus of the thalamus. Dorsolateral
reciprocal connections with posterior parietal area and the superior temporal sulcus.
➢ Decisions on spatial functions, object recognition, inhibition of behaviour, goal-directed
behaviour, self-knowledge, consciousness and working memory.

➢ Luria find did that the front prefrontal region serves as a tertiary zone for the limbic system as
well as the motor system. They have rich connections with upper part of the brainstem and thalamus
and all other cortical zones.

➢ Through the first set of connections the prefrontal areas particularly the basal and medial aspects
of lobe, are intimately concerned with the state of alertness of the organism,

➢ While the rich connections with the posterior receptor areas and motor areas allow the lateral
prefrontal regions to organize and execute the most complex of man’s goal directed or purposeful
activities.

➢ The division of prefrontal cortex into lateral and Basomedial regions may serve as a useful
approximation in search for brain- behavior relationships. This would accord well with non-
projections from the thalamus.

➢ The medial part of the dorsomedial nucleus projects to ventromedial aspects of the prefrontal
cortex while the latter part projects to the dorsal lateral aspect.
➢ With posterior frontal lesions adjacent to motor cortex the disturbances are of those organizations
of movement as relations move forward they lead firstly to disintegration of motor programs and
then to a disturbance or comparison of motor behavior with the original plan.

➢ All these disturbances are particularly marked in patients with lesions of the lateral zones of the
left (dominant) frontal lobe which are closely connected with the cerebral organization of speech and
disorganization of speech activity and those behavioral acts which are especially dependent on the
participation of speech for their regulation.

➢ Speech disorders are particularly associated with lesions of the lower part of the lateral cortex of
the dominant hemisphere.

➢ Disturbance of speech from lesions of the posterior inferior area broca’s area is termed as motor
aphasia.

➢ There are other speech disturbances offer more subtle nature found with lesions anterior to
broca’s area which are seen in patients who are not considered clinically to be suffering from
aphasia.

➢ Luria included such disorders of the left inferolateral cortex:

1) inability to make spontaneous discursive statement, 2)

difficulty in expressing a though in discursive speech and

3) verbal adynamia or frontal aphasia.

Basomedial portions of the Lobe

➢ Basomedial portion of the lobe controls:

➢ the state of activation,

➢ controls inhibition and

➢ emotional life of an individual.

Frontal Lobe Syndrome

• Changes in personality after brain injury are most often noted after damage to the frontal
lobes. These changes have been reported for well over a century and no description of what
has come to be called the frontal lobe syndrome would be complete without reference to the
case of Phineas Gage.

The following summary, which has appeared in many places, is quoted by Kimble (1963):

“Phineas P. Gage, an ‘efficient and capable’ foreman, was injured on September 13, 1848, when a
tamping iron was blown through the frontal region of his brain. He suffered the following change in
his personality according to the physician, J.M Harlow, who attended him. ‘He is fitful, irrelevant,
indulging at times in the grossest profanity (which was not previously is custom), manifesting but
little deference to his fellows, impatient of restraint or advice when I conflicts with his desires, at
times pertinaciously obstinate yet capricious and vacillating, devising many plans for future
operation which no sooner are arranged than they are abandoned in turn for others appearing more
feasible. His mind was radically changed so that his friends and acquaintances said that he was no
longer Gage”
• Such gross changes are usually seen only with severe bilateral frontal; damage, but the lack
of inhibition, impulsivity and lack of concern of such patients can be seen to a lesser degree
in others whose injury is less severe.
• In its milder examiner forms it could be taken to be within the extreme range of
‘extraversion’ if the examiner were not familiar with the patient’s prior personality. Another
of the characteristic features of the frontal syndrome is the mania for making puerile jokes
referred to as ‘Witzelsucht’.
• Many of these signs seen in the early months after head injuries fade with the passage of
time, though the more severe the injury the more likely it is that some personality change will
remain.
• Besides the all-too-common effects of road trauma, both war injuries and psychosurgery have
provided potent evidence of the personality changes which accompany bilateral frontal
damage.

• The complex set of changes with bilateral frontal damage which comprise the frontal lobe
syndrome was concisely expressed by Benton (1968). The first set of changes is related to
what may be loosely termed personality: ‘diminished anxiety and concern for the future,
impulsiveness, facetiousness and mild euphoria; lack of initiative and spontaneity. (However,
it may be noted that, while they are not mentioned prominently in group studies, anxiety
states have been described occasionally in clinical case reports as a presenting symptom in
patients with frontal lobe disease).’
• Related to complex impairments of motivation and social behaviour is the appearance of
stereotyped and ritualistic behaviour without signs of anxiety. This may sometimes be termed
obsessive-compulsive behaviour.
• A recent case following head injury (Donovan & Barry) highlights two features: firstly, the
likelihood that the symptoms may be more common than anticipated because they are not
reported b the patient due to apathy; secondly, as in similar cases, neurological and
neurocognitive examinations were normal.
• In 1993, Minski reported the symptoms with cerebral tumours, and one of the present authors
has seen two cases of frontal neoplasm where the ‘obsessional’ features had led to a first
diagnosis of OCD. Baxter’s 1992 review of brain imaging studies shows evidence in many
cases of lesions in the orbitofrontal cortex.
 • The second set of changes may be termed intellectual: ‘impaired integration of behaviour
over a period of time, a deficit which for want of a better term has been called impairment in
‘recent memory’; loss of the capacity to think in abstract terms; finally, inability to plan and
follow through a course of action and to take into account the probable future consequence of
one’s actions, a deficit which is perhaps closely related to some of the observed personality
changes as well as to the impairment in recent memory.
• There is some evidence from lesion studies both in animals and man that there is at least a
partial dissociation between the two major groups of symptoms. This is supported by the
extensive literature on psychosurgery suggesting that the intellectual changes are more
associated with damage to the dorsolateral connections, while the personality changes are
more associated with damage to the orbitomedial or basomedial regions.
• A third set of changes may be seen in association with lesions of the mesial aspects of the
frontal lobes. Since these latter are relatively well protected compared trauma but with
obstruction or haemorrhage of the anterior cerebral arteries which supply the region.
• The principal clinical feature is an Adynamia and this may evidence itself in a complete or
relative lack of verbal or overt behaviour.
• Thus the term frontal syndrome has broad connotations implying the presence of all three sets
of changes or some combination of subsets. Rather than refer to the frontal lobe syndrome it
might be preferable to refer clinically o the presence of a frontal lobe syndrome and to
specify which set of changes is prominent in the individual case.
• This specification might assist in pointing up the features, for example, which will require the
design of appropriate rehabilitation procedures. Other terms such as dysexecutive syndrome
which attempt to encapsulate a major part of the so called frontal features without begging
the question of their origin in frontal pathology have not yet found wide acceptance.
• This may come with further analysis in the discipline of cognitive neuropsychology.

Utilization Behaviour

• In 1983, Lhermitte employed this term to describe a form of behaviour in which patient with
a frontal lesion gave an instrumentally appropriate but exaggerated response to objects that
were introduced to them. At first this behaviour was described in terms of clinical
examinations but was later described in detail with two patients in a wide variety of
environmental situations.
 • In the presence of objects, and without direct instruction, the patients appeared constrained to
carry out sequences of behaviour commonly associated with them.
• Lhermitte et al (1986) examined 75 patients who showed utilization behaviour or its early
stage, termed imitation behaviour.
• A high proportion of these had involvement of the inferior part of the anterior half of one or
both frontal lobes, an area thought by some to be responsible for the lifting of control in
frontally damaged patients. The condition has been described in association with a number of
pathological conditions affecting this region, and also with thalamic lesions, suggesting that a
disconnection or diaschisis or deafferentation effect may be at work.
• Shallice and colleagues (1989) have made a distinction between induced utilization, where
objects are presented directly, and incidental utilization, where the objects are nearby through
the patient’s attention is not directed to them. These authors remind us that psychologists
have long been aware that a person’s behaviour may be affected by implicit as well as by
explicit cues.
• Orne (1962) termed these features the ‘demand characteristics’ of a situation. They clearly
influence the behaviour even of those without brain lesions.
• Patients with utilization behaviour appear to have an extreme form which Lhermitte (1986)
called the ‘environment dependency syndrome’. This author has recently described imitation
and utilization behaviour in major depression and patients showing under activity in the
frontal regions on PET scan.

Frontal Adynamia

• This condition is seen predominantly with bilateral lesions affecting the medial aspects of the
frontal lobes and hence may be seen most clearly with lesions of the anterior cerebral arteries.
It ranges from a mild state, where the patient is less active usual with little spontaneous
speech, to the fully blown akinetic mutism which accompanies bilateral anterior cerebral
artery infarction.
• In reporting three such cases, Freeman (1971) described that condition as ‘a disorder of
consciousness characterized by unresponsiveness but with the superficial appearance of
alertness. The patient’s eyes are open but he neither speaks nor moves, nor is the examiner
able to communicate with the patient’.
• The sister of one of our patients, noting the apparent alertness, felt that her brother simply did
not want to speak with her.
 • Akinetic mutism of frontal region is seen with bilateral cingulate gyrus lesions, but the
disorder is also seen with peri-aqueductal lesions.
• The milder cases may be described as placidity and those in our experiences have certain
common features. Patients are generally akinetic and they will remain in one situation for
long periods of time.

• No spontaneous conversation or comment is proffered, but they will reply cogently to others’
conversation and carry out actions without any difficulty, even when these are of a high level
of complexity.
• On several occasions we have found that, despite their Adynamia, patients may show a
normal reaction time even to a long series of signals occurring randomly over several
minutes.
• Frontal Adynamia presents a serious impediment to rehabilitation since, while patients may
respond normally to direct external stimuli, they lapse back into inactivity on their
withdrawal. The problem appears to lie in an inability to generate and sustain one’s own
motivation, a defect of the voluntary regulation of arousal brought about by a break between
the neocortical and brain stem components of arousal mechanisms.

UNIT 3: LESION STUDIES AND COGNITIVE CHANGES

ABSTRACT THINKING

• The various qualitative and quantitative changes in abstract thought processes due to damage
occurring within the cerebrum is of prime focus. Another issue to concentrate is on whether
we can attribute these qualitative or quantitative changes in abstract thinking processes to
frontal lobe involvement.
• Kurt Goldstein (Goldstein 1936a, b, 19391a etc.) put forth the notion that there exists two
different qualitative modes of thought and behavior: abstract and concrete. Accordingly, he
said that a normal person is capable of exercising both modes according to demands of the
situation – while many brain impaired patients are restricted to using only the concrete mode.
• Goldstein states two different principles to define the abstract-concrete dimensions of thought
processes. His central concept was that of – taking up the “abstract attitude”. Abstract attitude
and concrete attitude though initially postulated as a dichotomy, Goldstein states that there
exists differing degrees of abstraction and concreteness. In concrete attitude, we experience
and recognize a given thing or situation immediately on the basis of the presenting claims.
Whereas, the abstract attitude is separate in principle from concrete because it involves going
 beyond the present claims of sense or object impressions; specific properties or situations are
overlooked.
• We do not switch or gradually transition from one mode to another, rather abstract attitude is
a completely different process or activity of an organism.
• The greater difficulty connected with abstract approach is not merely one of greater
complexity involved in it (measured by the number of separate subservient functions
involved) – rather, it also involves demands of a different behavior of a new quality which is
different from behavior involved with concrete thinking.

Abstract attitude was defined by performance on a series of tests such as Weigl Colour Form Sorting
test, and an object-sorting test together with a block design test such as Kohs type and the
GoldsteinScherer Stick test.

The abstract attitude requires the following conscious and volitional modes of behavior:

1. To detach our ego from the outer world or from inner experiences.
2. To assume a mental set.
3. To account for acts to oneself; to verbalize the account.
4. To shift reflectively from one aspect of the situation to another.
5. To hold in mind simultaneously various aspects.
6. To grasp the essential of a given whole; to break up a given whole into parts, to isolate and to
synthesize them.
7. To abstract common properties reflectively; to form hierarchic concepts.
8. To plan ideationally; to assume an attitude towards the ‘mere possible’ and to think or
perform symbolically.

Quantitative versus qualitative changes

Reitan and colleagues (1955)

• Established marked differences between brain impaired and normal subjects on ten tests of
the Halstead Impairment Index and Wechsler-Bellevue Scale. He stated that if different kinds
of abilities were used by the brain damaged subjects, the interrelationships or correlations
between various tests would differ from the interrelationships between tests shown by the
group without brain damage. A correlation of 0.85 was obtained between the matrices for
brain-damaged and control groups. Thus, even though brain damaged individuals showed
 definite impairment, their abilities remained essentially of the same kind as non-damaged
subjects.
• Similarly, in other studies by Reitan (Reitan, 1959 – no marked differences between brain
damaged and non-damaged groups on Halstead Category test but differences in mean error
scores; Doehring & Reitan, 1962 – no marked differences found in the left hemisphere, right
hemisphere and control groups in the overall ratio of errors on subtest to total errors on the
Category test)
• So why were no differences observed between these groups in performance, if there are
deficits or impairments among brain damaged subjects? The answer is that according to
Goldstein G (1968), the qualitative position adopted by Goldstein and the quantitative
position assumed by Reitan has been the failure to consider the notion that BOTH
observations maybe correct depending on the kind of brain-damaged patients and the abilities
being evaluated. Bourne (1966) states that the issue of qualitative versus quantitative
impairment of abstract reasoning in brain damaged patients is NOT independent of other
factors related to the patient itself such as ag, type of deficit in the patient, locus of lesion and
the problem to be solved. While some patients cannot adopt the ‘abstract attitude’ (postulated
by Goldstein), others behave quite normally (as observed by Reitan in his studies to assess
the quantitative dimension of abstract thinking) indicated by the failure to find a marked
difference in performance on abstract thinking tests.

Frontal locus and abstraction

• Patients with frontal lesions perform quite poorly on tests of abstraction. Some of these
studies involved patients who had lesions made on them using psychosurgery while other
studies pointed out that lesser losses maybe observed when less radical surgical operations
such as psychosurgery were done on patients performing on abstraction tests.
• Examination of cases with other frontal lesions including excision of small and large portions
of the frontal lobes has described poor abstract ability defined by poor performance on one or
more tests. But the main issue of such studies are that, they have concentrated on the
behaviour of individuals with frontal lesions without any comparable examination of patients
with lesions in other locations, or studies where this comparison has been done. One notable
example has been of the Halstead studies where evidence of such major differences between
frontal lobe lesioned patients and non-lesioned patients is unconvincing.
 • The major reason for such unconvincing findings lies in the nature of the tests being used to
assess abstraction abilities (for example, problems with the Halstead tests) as well as the
unwarranted assumptions which are made about the abilities felt to be central to the solution
(When infact, it isn’t so!)
• A patient can fail on a test of abstract thinking for a number of reasons other than alteration
in the ability to think abstractly. This view is supported by Milner – who argued that the
frontal patient may fail on a test of abstract thinking possibly because of an inability to inhibit
preferred modes of responding. This is further supported when we examine tests used to
assess abstract thinking function – for e.g., the Halstead Category test, which is arguably a
very complex test on which most brain damaged individuals fail due to a there being several
factors crucial for good performance on the test. If all these criteria are not fulfilled,
unavoidably a patient stands good chances of failing on the test and it may not be accounted
for by frontal lesions or functioning.

PLANNING AND PROBLEM SOLVING

• Luria’s contributions to neuropsychology focuses on conception of frontal lobes at the

summit of the brain’s hierarchy.
• Luria states that man does not merely react passively to incoming information – rather he
creates intentions, forms plans and programmes of his actions, inspects their performances
and regulates his behavior so that it conforms to these plans and programmes and finally, he
verifies his conscious activity, comparing the effects of his actions with the original intentions
and correcting any mistakes he has made.

• Thinking is defined as a form of problem-solving behaviour which involves the correlation

and integration of critical events in time and space. It is characterized by (a) a period of
preliminary exploration, (b) a pre-solution period of search, (c) a period of vicarious testing
of tentative solution, (d) an act of closure and registry of a memory trace, (e) appropriate
action - definition by Halstead (1960). Each of these steps are dependent on the integrity of
the frontal lobes. Some of the complexity in functioning of the various frontal lobe regions
are gained from an examination of three areas:
Maze behaviour

• Nearly in 1914, Porteus introduced a set of paper and pencil maze problems which were
widely used, and the reason for its popularity was because it measured characteristic signs of
man’s intelligent behaviour: such as ‘planfulness’, ‘planning capacity’ or at another time as
‘prehearsal’ that is known as a mental rehearsal of the act that the individual is about to
perform. Porteus considered planning as a prerequisite to every intelligent act (Porteus, 1950,
1958) and that it was a factor maximally represented in the frontal lobes. Loss of right or left
frontal lobes through frontal lobectomy results in detectable impairment of those mental
processes which are requisite to planned initiative.
• Patients undergoing classical lobotomy procedures showed a clear loss in this area of
planfulness whenever measured using mazes. Importantly, the losses on the Maze test
depended on the location of the lesion within the frontal lobe as shown by Crown (1952) and
Lewis and colleagues (1956) – posterior and superior lesions of the frontal lobe affected
performance more consistently than did lesions in other sites. Whereas Robinson’s studies
suggested that the extent of loss maybe related more to the extent or amount of the frontal
lobe that has been disconnected.
• Another notable type of maze drawing test sensitive to cerebral impairments or frontal lobe
dysfunction was introduced by Elithorn (1955), but it was shown in later studies that the
Elithorn Maze Test (involved several dots and the subject is asked to draw on the dotted lines
and find a way from the bottom to the top, passing through the maximum number of dots
without ending up drawing a ‘diamond shape’ or reversing directions) was not helpful in
showing differential impairment between the frontal patients.

Visuo-constructive activities

• The Kohs Block Design test has been used to assess brain impairment through the measure of
visuoconstructive abilities. Although a popular addition to tests such as the Wechsler scales,
it failed to live up to expectation as a universal indicator of brain damage but recent
reappraisal of the Block design test has shown that patient’s performance on the test maybe a
valuable indicator of brain impairment when the lesion is situated in the frontal or parietal
regions.
• Constructional difficulties are also sometimes known as constructional apraxia and in
posterior lesions, the difficulties arises because of a loss of spatial organization of the
elements. Luria and Tsvetkova (1964) demonstrated that frontal constructional difficulties
 arises through the disruption of one or more of the steps mentioned initially – namely,
intention, programming, regulation or verification.
• An example for the point made by Luria and Tsvetkova (1964) arises from the fact that brain
damaged patients with frontal lobe issues can perform much better on tasks such as Kohs
Block design by facilitating a programme for reaching the solution to solving the Block
Design task (or generating part of the solution). When such a programme is provided, the
patient could immediately solve the puzzle and without any errors when presented with a
model or design.
• Lhermitte et al employed the Complex Figure of Rey – and frontal lobe lesioned patients
showed much better performance when they copied the figure, compared to being asked to
reproduce the entire figure from memory. This was not caused due to a primary disorder of
the memory rather, after it was noted that such patients showed poor reproduction from
memory, these patients were given a structured sequence of the figure to copy (they copied
each figure of the complex figure individually, slowly until the entire sequential programme
for its execution was experienced by them on a number of different occasions). This was
followed by asking them to reproduce from their memory and they showed more superior
performance than the trial before the programme facilitation for reaching the solution, or the
‘copying’ task was provided. The improvement occurred due to the facilitation of the
programme or the provision of it rather than sheer weight of practicing.

Arithmetical problem solving

• With frontal lobe lesions, there do not appear to be significant disturbances of wellestablished
operations such as addition or subtraction and none of the ‘spatial’ mathematical difficulties
seen in parietal patients. The essential difficulties are better described as those of problem
solving or of ‘discursive intellectual activity’.
• Once again – patients may be able to repeat a problem but their actions suggest a form of
amnesia. They seem to have forgotten how to generate and execute even a simple two or
three step programme which would have presented no difficulty prior to their lesion. As in
other areas of behavior much of their difficulty arises through incomplete analysis and an
inability to inhibit the first tendency aroused by the problem.
• Christenen (1975) gives a summary of the behavior of a frontal lobe patient confronted by
arithmetical problem solving: Patients grasp only one particular fragment of the problem;
they do not make any plans but starts to carry out disconnected arithmetical operations with
 this fragment. The whole process of solution may be transformed into a series of impulsive,
fragmentary arithmetical operations frequently unconnected with the ultimate goal.
• Another problem solving task that is poorly performed by frontal lobe patients compared to
normal control groups is known as ‘cognitive estimation’ (Shallice & Evans, 1978).
Cognitive estimation basically means generating a plan and executing it while checking it
against their store of common knowledge (some examples of such questions requiring
cognitive estimation are ‘What is the length of an average man’s spine?’, ‘What is the length
of a five pound note?’ etc.) Such questions require a reasonable or approximate estimation
which is what is usually seen among normal patients but frontal lobe lesioned patients show
wildly exaggerated or grossly inappropriate estimations. Similarly, frontal subjects have also
shown difficulty in estimating the frequency of recurrence of items in a presented series, right
frontal subjects having more difficulty than left. The authors consider the difficulty occurring
due to poor memory searching, poor cognitive estimation or both.

Error utilization

• One of the major changes in behavior with frontal lobe cases is the apparent lack of
awareness of deficits. Luria has referred to this as a ‘lack of criticism’ or ‘lack of critical
attitude towards one’s own actions’ (Luria & Homskaya, 1963, 1964) and felt that this could
be regarded as the result of a general loss of some feedback mechanism, a disturbance in
signals of error or an inadequate evaluation of the patient’s own action.

• In discussing their patient’s inability to carry out compounded or symbolic instructions,

Luria, Pribram and Homskaya (1964) stress what has been pointed out several times already,
namely that the failure is not one of understanding what is required, “these incapacities seem
not to depend on any difficulty in apprehending the instructions per se: they may however, be
related to an instability, shown by our patient to evaluate errors, especially self-produced
errors.
• A most important contribution to conceptualizing the nature of the frontal defect has been
made by Konow and Pribram (1970) – they describe a patient who made errors and at the
same time gave clear indication that she was aware of making them. She was not, however,
able to correct them. We therefore confronted her with a test in which one of us carried out
the commands of the other, sometimes correctly and sometimes erroneously. The patient
usually had no difficulty spotting our errors. This was true even when they were embedded in
rather complex serial performances.
 • The important feature was the patient’s inability to use such information to modify her
ongoing programme of action. This analysis leads to an important distinction which should
have marked implications for attempts to rehabilitate patients with frontal injury, i.e., the
distinction between error recognition and error evaluation on one hand and error utilization
on the other. The latter term is a clear expression of the dissociation between thought and
action mentioned by many neuropsychologists in a variety of ways e.g., the ‘dissociation
between knowing and doing’. One study has pointed to the similarity of such behavior with
that of non-human primates with frontal damage (Rolls et al., 1994).
• Error utilization is sometimes overlooked and can be very disruptive unless appropriate tests
are used. It is commonly seen in as a disability among individuals with closed head injury
and in chronic alcoholism. Tests such as Serial maze learning of the length and complexity of
the Milner pathway (Milner, 1965) using the Austin maze (Walsh, 1991) or similar apparatus
brings out the difficulty very well. It must be noted that in such tests, sometimes the subject
may rapidly learn the general plan of the maze, reducing errors to a small number in a few
trials only to have difficulty in eradicating errors completely. Even when the subject has
reached an error-less trials, subsequent trials may show recurrence of errors. This highlights
the point that measures such as trials to criterion (e.g., one errorless trial) maybe quite
misleading as a measure of the patient’s capacity to work effectively. Whenever there is
suspicion of frontal lobe involvement the criteria of learning should be the execution of a
stable error-free performance.

• Luria argued that the basic factor contributing to the frontal patient’s difficulties was lack of
the ‘verbal regulation of behavior’. In other words, the patient’s verbalizations (both internal
and external) do not command their actions.

Tower of London problems

• This set of problems is the first of a series of tasks intended by the originator (Shallice, 1982)
to examine frontal lobe processes. It consists of a series of novel problems of graded
difficulty in which the most efficient solution depends on breaking down the goal into
subgoals which must then be tackled in an appropriate order. By arranging the coloured beads
on sticks, the subject must achieve a given arrangement in a state number of moves by
shifting the beads one at a time from stick to stick.
• In the seminal study, patients with left anterior lesions showed a specific deficit compared
with right anterior and both posterior groups. In another study of normal subjects on the test
 employing single photon emission computerized tomography (SPECT) showed not only an
increased perfusion in the left prefrontal region, but also a higher measure in those who took
more planning time for their moves (Morris et al., 1993), while another SPECT study with
volunteers showed bilateral frontal activation with the Tower problems. The tower problems
seem more highly loaded on a planning factor than most others in common use and are a
welcome addition to research and clinical method.
• While frontal patients undoubtedly perform badly on planning tests, Goel and Grafman
(1995) suggests that the difficulty may not be one of ‘looking ahead’ as is often assumed but
may result from a difficulty in resolving goal-subgoal conflicts which arise in the
performance of these tasks.

RIGIDITY AND CEREBRAL IMPAIRMENTS

One of the most frequently mentioned features of the behavior of brain impaired patients is a lack of
flexibility in their approach to many situations – in both clinical and psychological test situations.
Particular studies express this in different ways:

1. Lessening of the ability to shift from one concept to another when compared with normal
subjects.
2. Inflexibility, rigidity, perseveration or stereotyped behavior.
3. Sensitivity to the effect of set or einstellung.
• This list is by no means exhaustive but points to the common occurrence of a mode of
behavior we will term inflexible. This condition or descriptions are not a unitary disability
and has been associated frequently in tests of abstract thinking as well test situations
involving learning and problem solving as well as being reflected in the patient’s everyday
behavior. Among the various tests which have brought out the brain impaired subject’s
inflexibility – prominent is the Goldstein-Scheerer tests, parts of the Halstead battery, the
Wisconsin Card Sorting test, stylus-maze problems, pegboard discrimination, paired
associate learning, problem solving and word association tests.
• One fruitful experimental approach that has received little attention is to examine flexibility
under the paradigm of negative transfer. The only direct examination appears to be that of
Gleason (1953), who defined negative transfer as ‘retardation in the acquiring of an activity
as a result of being engaged in a prior activity’. Gleason found support for an increase in
negative transfer in brain damaged subjects on several tasks for example, a pegboard
discrimination task, a card sorting task and a rotated stylus maze, demonstrating that there
 was ‘an application of old responses in new situations where such responses are
inappropriate’.
• There is considerable evidence that inflexibility is particularly marked after lesions of
prefrontal regions. Milner’s findings (1963, 1964) suggests that such deficits maybe more
associated with lesions affecting the dorsolateral aspect of the frontal lobes rather than the
orbitomedial regions. Such a finding maybe in keeping with the reports of psychosurgical
studies which show little or no intellectual alteration when division of fibre pathways is
restricted to the ventromedial quadrant of the frontal lobes. However, a recent study of
patients with ventral damage to the frontal lobes showed a tendency to continue to make
inappropriate responses on discriminative tasks to a previously rewarded stimulus (Rolls et
al., 1994).
• Despite the accentuation of the frontal lobe, there is clear evidence that inflexibility is also
found with lesions in other parts of the brain. An example of inflexibility is exhibited in
perseveration that is not merely an accompaniment of aphasia but is found in a majority of
aphasic cases. The perseveration was linked to each patient’s specific deficit, according to a
study by Allison and Hurwitz (1967) where 16 of 24 patients studied by them showed
perseveration, and these deficits were elicited by tests related to the deficit but not by tests
related to preserved language functions. Like many authors they comment that perseveration
was facilitated by anxiety, but it sometimes also occurred in its absence. They also noted that
in their series, absence of spontaneous talk was a frequent accompaniment of perseveration.
Perseveration was seen in studies by other researchers as occurring in frontal lobe lesions,
temporal lobe lesions and even parietal lobe lesions.
• Perseveration or inflexibility appears to be most prominent when the task is difficult or the
patient is fatigued. This finding is not surprising considering the same conditions are relevant
when such lapses occur among normal subjects as well. It is when it becomes constant and
pervasive that it provides one of the most valuable signs of cerebral impairment.
• In some other studies, Luria has provided evidence of motor perseveration with frontal lobe
damage (Luria, 1963, 1965) and hypothesized two kinds of ‘frontal’ perseveration associated
with two different neuronal systems. The first type involves compulsive repetition of a
movement that has been initiated but the patient is able to shift from one action to another.

The second type represents what Luria terms ‘an inertia of the programme of action itself’ –
that is, the patient having once performed the required task is incapable of switching to the
fulfilment of any other task but continues even when instructed otherwise to perform the first
 task on which he has ‘stuck’. Type 1 is thought to be associated with deep seated lesions of
the premotor zones involving the subcortical ganglia while the second type is associated with
massive involvement of the anterior or basomedial portions of the frontal lobes. The only
significant difference is that, whereas in lesions of the premotor zones the pathological inertia
extends only to the effector components of the action, and performance of the programme as
a whole is undisturbed, in massive lesions of the frontal lobe it extends to the scheme of the
action itself with the result that performance of the programme becomes impossible.

• The validity of Luria’s ‘premotor syndrome’ as a separate distinct entity has been strongly
supported by the study of Derouesne (1973) of five cases of frontal tumor restricted largely to
the rolandic/prerolandic area. Luria makes it clear that patients who perseverate when given a
verbal instruction do not fail through lack of understanding but because the verbal instruction
does not regulate their behavior as it does with normal subjects.
• The question of inflexibility has also been explored through the question of abstract thinking.
A striking confirmation of this disability or difficulty which frontal patients have with
conceptual shifts was provided by Milner (1963). Using the Wisconsin Card Sorting test
(WCST) she examined patients with static lesions in the frontal and temporal regions before
and after cortical excision fort the relief of epilepsy. Patients with dorsolateral frontal
excisions were more impaired in their ability to shift than those with temporal excisions or
those with orbitofrontal excisions even when combined with temporal lobectomy. There were
no laterality effects shown in this study but a long term follow-up (Milner, 1975) showed
more lasting and consistent deficits on the WCST after left frontal lesions than with right,
such deficits being dissociable from language functions.

Intellectual loss and inflexibility

• It has often been inferred that intelligence and behavioural inflexibility maybe more closely
related among brain impaired subjects than among normal controls. Mackie and Beck (1966)
examined this proposition in a study employing 20 brain damaged subjects and an equal
number of controls. Their results showed that brain damaged subject may suffer intellectual
loss without increased inflexibility and that inflexibility can occur in brain impaired patients
without general intellectual loss.
• No examination of test inflexibility in patients would be complete without taking into
consideration factors which have been shown to influence the nature, extent and frequency of
such behavior in normal subjects. Goldstein (1943) in offering a comprehensive treatment of
 the problem of rigidity, emphasized that it is a normal phenomenon and distinguished two
types: 1) primary rigidity where the stimulus aroused a response system so strongly that the
individual became incapable of altering response set; and 2) secondary rigidity when the
subjects were faced with a situation with which they could not cope and which enabled them
to avoid difficult tasks which gave an overwhelming feeling of helplessness – the so-called
‘catastrophic reaction’.

• Goldstein’s stress on the fact that rigidity simply becomes exaggerated with brain damage
seems to refer to both these reactions. If these two areas are to be evaluated, then not only
must we study traditional learning theory which examines factors influencing primary
rigidity, but also study the effects of the individual’s perceptions of their successes and
failures as exemplified in studies such as those of Feather (1966).

Inflexibility and general considerations

Factor analyses of various rigidity scales and other personality, perceptual and aptitude scales
suggests that rigidity cannot be represented by a unitary factor. This multidimensionality of the
concept of rigidity is discussed by Chown who cites the following dimensions:

The difficulty with which old established habits maybe changed in the presence of new demands
(Cattell & Tiner, 1949).

The inability to change one’s set when the objective conditions demand it (Rokeach, 1948).

Resistance to shift from olds to new discriminations (Buss, 1952).

Lack of variability of response (Werner, 1946).

• Chown points out that while tests of rigidity can be classed under general headings e.g., (i) tests
of Einstellung or set, (ii) concept formation tests, (iii) tests of personality rigidity or
dispositional rigidity, (iv) tests of perceptual rigidity there is a need for a study of the
relationship between tests in these areas, especially as a good deal of overlap between some
measures is apparent.

FRONTAL AMNESIA

• There occurs a certain level of frequency of memory impairments in patients with tumors of
the frontal lobes and it is generally considered to be marked for recent memory. There is
 relatively little information about the presence of memory disorders with regard to relative
location of the lesion within the frontal lobe.
• It seems possible that some of the memory defects seen with frontal lesions maybe caused by
encroachment on connections between the frontal regions and the limbic or ‘axial’ structures.
Certainly some patients with frontal lesions appear to have difficulty with learning new
verbal paired associates, though a true Korsakoff type amnesia is rare (Hecaen, 1964).
• Some writers consider that there is no true amnesia in frontal patients i. e., no inability to
register or to retrieve material given the proper conditions. In their views, the memory
disorder is only apparent and the poor performance of frontal patients on some memory task
is better seen as a disruption of complex forms of behavior which reflects itself in numerous
ways.

• Luria was one of the foremost advocates of such a theory ‘a lesion of the frontal lobes leads
to gross disturbances of the formation of intentions and plans, disturbance of the formation of
behaviour programmes and disturbances of regulation of the mental activity and the
verification of its course and results.’ In other words, while leaving the operative part intact,
it leads to a profound disturbance of the whole structure of human conscious activity. Thus
the features of ‘frontal amnesia’ can be readily distinguished from the amnesic disorders
associated with temporal lobe lesions.

• In these latter cases, the whole programme or general meaning is preserved so that ‘patients
who are unable to retain separate elements often can grasp the general meaning of a sentence
or paragraph’. Frontal patients, on the other hand, suffer from a change in the total structure
of behaviour, which Luria (1971) believed was due to ‘high distractibility on one hand and
pathological inertia (of traces) on the other’, resulting in a loss of programmed forms of
activity.
• In such cases, there is no true amnesia, general or partial, and good retention of a series of
items in any modality after ‘free’ intervals of two minutes or more is seen. The defect of
retrieval in these patients results from an inability to create a stable intention to remember
with failure to “shift” their recall from one group of traces to another (Luria, 1971).
• Other writers have commented on the atypical nature of the memory difficulty of the frontal
patients; Benton (1968) calling it ‘impaired integration of behavior over a period of time, a
deficit which for want of a better term has been called impairment in “recent memory”.
Barbizet (1970) agreed that simple registration and recall of both visual and verbal material
was largely unaffected by ‘frontal lesions’.
 • In fact, only by means of memory tests in which the frontal patient must retain several facts
simultaneously before he can accomplish a specific task are difficulties in recall and learning
revealed, and these are often severe (according to Barbizet, 1970, p. 83). The frontal patient
while possessing the information necessary to solve a problem, often acts as if the (correct)
way to proceed has been forgotten.
• Barbizet considered the frontal memory defect to be of a specific type, affecting particularly
the use of previously acquired information. There is also a great similarity to the description
of the disorder given by Luria. The evidence seems to suggest that frontal lesions suppresses
the programs that governs the execution of the mental strategies that bring recall and
memorization into play during the operation of any new task, whether it be the resolution of a
problem or the learning of a piece of poetry.
• Frontal patients seem to have difficulty with voluntary learning or memorization but when
they are made to repeat material frequently by the examiner, they show that they are quite
able to acquire new information which they can also retain.

VERBAL BEHAVIOR

Two major forms of aphasia occur with frontal lobe lesions. These are Broca’s aphasia and
transcortical motor aphasia. There are also certain verbal disorders which maybe observed in the
frontal patient who is not demonstrably aphasic and which should be familiar to the
neuropsychologist.

Repetition and verbally directed behavior

Patients with lesions of the left frontal lobe often appear to have intact speech on superficial
examination, however, careful examination will often bring out evidence of perseveration, or in
severe cases, echolalia.

Patients typically have no difficulty in repeating isolated words or simple sentences and may also
manage quite well with an unconnected series of words but often have much more difficulty if the
word order is changed, e.g., they may repeat the set CAT-FOREST-HOUSE but have difficulty with
the rearranged set CAT-HOUSE-FOREST and FOREST-HOUSE-CAT, showing perseveration of
the earlier order. When asked to name objects the patient will name single objects readily but may
have difficulty if the objects to be named are presented in various pairings, eg., the series
WATCHPEN, SCISSORS-THERMOMETER, WATCH-THERMOMETER, SCISSORS-PEN may
produce
perseveration of earlier elements as the test proceeds. Such difficulties are exaggerated if the pairs
are presented with only a small temporal separation.

Difficulties in verbal regulation of behaviour was brought out most clearly when a sequence of
behaviour is called for, as indicated within the examples given below:

1. The patient is instructed to place in a line one black counter followed by two white counters
and to continue doing this. Two attempts from a patient with a left frontal meningioma are
shown below
B.W.W.W.B.B.W.W.W.W.W.W…
B.W.W.B.W.B.W.W.B.W.
2. Particular difficulty may arise where there is apparent conflict in the instructions, for
example, “Tap your hand once on the table when I tap twice and tap twice when I tap once”.
The patient maybe unable to do so or may begin correctly onto to deteriorate rapidly into
producing a random series or more commonly, a rigid stereotype of tapping irrespective of
the number of taps given by the examiner. However, there is no difficulty with echopraxis.

Verbal fluency

Frontal patient’s behaviour is often characterized by a general lack of spontaneity and voluntary
action to which the term ‘adynamia’ has been applied. There is frequently an associated
impoverishment of spontaneous speech and a reduction in the patient’s conversational replies which
often shrinks to passive responses to questions. These responses often have an echolalic quality, e.g.,
‘Have you had your lunch?’ “Yes, I’ve had my lunch.’ This verbal adynamia is often more marked
after left prefrontal damage than elsewhere in the brain including the right frontal region. Luria
(1973) felt that this form of reduction of speech should not be regarded as an aphasic disorder.

Milner (1964) employed Thurstone’s Word Fluency Test in a comparison of left frontal, right frontal
and left temporal lobectomies. This test requires the patient to write as many words as possible in
five minutes which begin with the letter S and then as many four-letter words which begin with C.
Milner found that left frontal cases were much poorer than the other two groups. Since there was a
marked difference between the left frontal and left temporal groups the effect would appear to be
specific to the left frontal region and not due solely to involvement of the left (language dominant)
hemisphere. Moreover, she found a double dissociation between the left frontal and left temporal
groups on the task of verbal fluency versus two tasks of verbal recall. Left frontal patients performed
poorly on verbal fluency but adequately on verbal recall of prose passages and paired associated
while the reverse was true of the left temporal patients.

Benton (1968) confirmed and expanded these findings. His test of verbal associative fluency has
become part of the Standardized Neurosensory Center Comprehensive Examination for Aphasia
(Spreen & Benton, 1969). The patient is asked to say as many words as possible in one minute for
each of the letters – F, A and S with the proviso that names and other capitalized words are to be
avoided and words with different endings but the same stem are not acceptable, e.g., eat, eaten,
eating etc. Benton restricted his examination to the frontal regions, the lesions being mainly tumours
classified in three groups, left frontal, right frontal and bilateral. On the verbal fluency test, left
frontal and bilateral cases were both inferior to right frontal cases, though there was no significant
differences between the first two groups. Benton pointed out that the left hemisphere patients were
‘ostensibly non aphasic’ and felt that, since the impairment was seen in speaking as well as writing, it
was a rather general higher level language loss. Ramier and Hecaen (1970) confirmed the verbal
fluency loss with left frontal lesions. They felt that the defect depended on ‘the interaction of 2
factors: frontal lobe damage (defective initiation of an action) and left-sided lateralization of the
lesions (verbal domain)’. There is now strong support for the usefulness of scores on verbal fluency
tests as an indicator of frontal lobe dysfunction.

Anterior alexia

• This term has been used to describe what some consider to be a separate syndrome (Benton,
1977). Benton (1977) used the term the third alexia to distinguish it from the other two major
forms which occur with centrally placed and occipital lesions respectively. Patients with
anterior alexia most often have a non-fluent aphasia and right sided hemiplegia or
hemiparesis. Other signs of posterior involvement, such as hemianopia and auditory
comprehension difficulty, are characteristically absent.
• Anterior alexics may be able to read some words, particularly nouns and action verbs, but are
unable to read some words such as prepositions and terms of relationship, which are
syntactically important. They are also unable to read single letters even where they can read
the whole word. Thus, this form of reading difficulty has been termed as syntactic or literal
alexia.
PERCEPTUAL DIFFICULTIES

• In 1861, Aubert described the following phenomenon which bears his name. When subjects
are asked to align a luminescent rod to the vertical in a dark room they are usually able to do
so with a fair degree of accuracy. However, if the subject’s head (or both head or body) is
tilted the perceived vertical is displaced to the side opposite to the direction of body tilt and in
proportion to the degree of head or body tilt.
• Teuber and Mishkin (1954) examined the Aubert effect in subjects with penetrating missile
wounds in various locations. They found a ‘double dissociation’ on this task with regard to
anterior (frontal) and posterior (parieto-occipital) lesions. Frontal patients had difficulty in
setting the rod to the vertical with their head and body tilted (the visual-postural condition).
Posterior lesion subjects had little difficulty on this task. On the other hand, posterior subjects
performed much more poorly on a visual-visual condition where they were required to set a
black thread to the apparent vertical against an interfering background of visual stripes while
their body was upright. On the simple visual condition, for setting the rod and also on a
simple postural task where patients had to set their own chair to the vertical there were no
major group differences.

Visual search and analysis of complex material

• Teuber’s laboratory was also the first to demonstrate experimentally a ‘subtle but lasting
deficit in visual searching’. Patients were required to point as quickly as possible to the
matching stimulus when one of the 48 stimuli was displayed on a circular centre area. Frontal
patients were much poorer on this task than either control subjects or non-frontal lesion cases.
Unilateral frontal cases, unlike the other subjects, also were disproportionately slower in
finding objects on the side opposite the lesion.
• An analysis of eye movements in frontal subjects appeared in a number of publications. Luria
et al (1966) examined eye movements in a patient with a large right frontal tumor and
demonstrated a disturbance in the complex process of exploration of complex pictures.
• Chedru et al (1973) reported increased identification time for pictures in the contralateral
visual half-field but found that the searching activity was similar in the two visual half-fields.
They considered that the longer identification time might be accounted for by assuming that
some alerting value was lost on the affected side by an alteration or disruption of
occipitofrontal connections, an explanation similar to the disconnection hypothesis invoked
elsewhere. Eye movements of the four patients studied were normal on verbal command and
 in following a moving target but differed from normal when complex pictorial material was
used.
Reversible perspective

• Cohen (1959) working in Teuber’s laboratory with penetrating missile cases, found that
unilateral frontal cases experienced far fewer reversals of a double Necker cube than patients
with lesions in other areas, though all unilateral brain damaged individuals were inferior to
controls.
• The average number of reversals was smallest for the right frontal group. Rather
unexpectedly, bilateral frontal cases showed the reverse phenomenon, reporting many more
reversals than normal control subjects. The significance of these findings is uncertain.

UNIT 4: Laterality and the frontal lobes

 As far back as Jackson (1874), some writers remarked on the greater disturbance of
behaviour by left frontal lesions than by right, though some studies, e.g. Rylander (1939),
have found no difference associated with the laterality of lesion.
 Zanglwill (1966) pointed out that Feuchtwanger and Kleist had described certain higher level
verbal difficulties in left frontal lesions which were not seen in right-sided cases. In
summary, these were:
(1) A certain loss of spontaneity of speech in the absence of articulatory disorder
(2) Difficulty in evoking appropriate words or phrases, amounting on occasions to frank
agrammatism.
(3) In some cases, definite impairment of verbal thought processes.
• More recently, attention has been addressed to the question of whether the asymmetry of
function of the two hemispheres is reflected in the frontal tumor cases by Smith (1966), using
the Bellevue-Wechsler scale, appeared at first to agree with Teuber’s evidence that frontal
lesions have less effect on test intelligence than lesions in other areas. 31 frontal cases had a
mean IQ of 95.55, whereas 68 posterior cases had a mean IQ of 91.46.
• However, a division into left frontal (14 cases) and right frontal (17 cases) revealed a
significant mean IQ difference between the left (90.1) and right (99.5) cases. Smith
reexamined Pollack’s data on the Wechsler-Bellevue scale for the latter’s reported tumour
cases and found a mean IQ of 86.25 for 4 left frontal cases and a mean IQ of 103.5 for 6 right
frontal cases. Thus, by grouping together all frontal cases, on important difference had been
concealed.
 • Smith also examined the ages of left and right frontal cases reported in several studies and
found consistently younger ages for those with left frontal tumors. Together with clinical
evidence that left frontal tumors appear to declare themselves earlier than right frontal
tumors, Smith finds this a strong argument against the proposition that the disruptive effect of
frontal tumors can be attributed to their larger size since the most disruption clearly comes
from those of earlier onset, i.e. left frontal.
 Benton (1968) looked at the question of laterality effects in frontal lesions by utilizing tests
meaningfully related to evidence of hemispheric asymmetry. The following tests were used:

Two ‘left hemisphere’ tests: (i) verbal associate fluency and (ii) paired associate verbal learning

Two ‘right hemisphere’ test: (i) three-dimensional constructional praxis test and (ii) copy designs
(Benton visual retention test)

Two ‘bilateral’ tests: (i) Gorham proverbs and (ii) temporal orientation

 Although only a small sample was available, the findings strongly suggest that
interhemispheric differences are reflected in the frontal lobes. Left frontal patients were
inferior to right on the word fluency test, while right frontal patients were inferior to left
frontal on both the constructional praxis and copying tasks. Somewhat unexpectedly, the
right frontal group was inferior to the left on the proverbs task.
 While the data on verbal fluency has been interpreted as showing poorer performance by left
frontal cases, Jones-Gotman and Milner (1977) found a right frontal bias on their test of
design fluency in which subjects had to generate nonsense designs. The nature of the task and
particularly its evaluations had made this finding difficult to replicate.
 The care that must be taken in the unequal test performance of two lesion groups is shown by
the experiments on recency judgements carried out by Milner’s group. Two early studies
(Milner 1971, 1974) asked subjects to judge which of two stimuli inserted in a series had
been presented more recently.
 Patients with left frontal lesions were impaired on verbal material but not on non-verbal
material (representational drawings and abstract designs), while those with right frontal
lesions were impaired on all three tasks, their performance on the verbal task is being nearly
poor as the left frontal group.
 Similarly, Ladavas et al (1979) found a material specific recency effect for patients with
epileptic foci in the left frontal region.
  In a second set of experiments (Milner 1982) despite the use of similar material, there was a
strong bias towards poorer performance by the left frontal group when subjects were required
to choose their own order of responding. The findings comports well with data which argues
for a greater role for the left hemisphere in planning and programming behaviours.
 The recent study of Wiegersma et al (1990) supports Milner’s propositions that frontal
subjects are deficient on subject-ordered tasks.
 Finally, it is not inconsistent with clinical findings that patients with left frontal lobectomy
demonstrate impulsive behaviour on a cognitive risk-taking task, and are impaired in solving
such tasks. (Milner 1992).

MODULE 4: FUNCTIONAL ASPECTS OF TEMPORAL LOBE

Unit 1: Basic Anatomy of temporal lobe

• The temporal lobe lies below the lateral cerebral fissure or fissure of Silvius. The lateral
surface is divided into three convolutions or gyri (superior, middle and inferior gyri) by two
sulci. The superior temporal sulcus runs approximately parallel to the lateral cerebral fissure,
beginning near the temporal pole in front and running back until, near its end, it turns upward
for a short distance into the parietal lobe where it is surrounded by the angular gyrus.
• On the inner portion of the lateral sulcus the cortex of the superior temporal gyrus dips into
the insula in several short horizontal convolutions known as the inferior transverse gyri of
Hecht. The middle temporal sulcus, which is often in two disconnected parts, divides the
middle from the inferior temporal gyrus, a portion of which lies on the inferior or basal
portion of the lobe.
• The posterior boundary is formed by an imaginary line joining the parieto-occipital sulcus to
the pre-occipital notch, and the superior boundary runs backwards from the upper end of the
lateral sulcus to join the posterior boundary at right angles. Thus, the temporal lobe merges
into the visual cortex behind and the inferior parietal lobule above. The inferior parietal
lobule is made up of the supramarginal and angular gyri. The supramarginal gyrus surrounds
the ascending branch of the lateral sulcus.
• The inferior parietal lobule lies at the confluence of the parietal, temporal and occipital lobes,
i.e with those posterior or retrorolandic portions of the cerebral cortex that are concerned with
the various sensory systems of the body, as this area is rich in multisensory connections, it is
concerned with the integration of sensory information. Lesions in the lobule, particularly in the
 dominant hemisphere give rise to symptoms which are characteristically different from lesions
in other areas.
• Whereas lesions in the primary projection areas and the association areas which surround
them are modality specific, giving deficit in only one sensory system. Lesions in the
supramarginal
and angular gyri disrupt ‘the mnemonic constellations that form the basis for understanding
and interpreting sensory signals which are based on multisensory perceptions of a higher
order’.
• The inferior surface of the temporal lobe is also divided into three major gyri. Part of the
inferior temporal gyrus occupies the lower lateral aspect oof the lobe and is separated from
the fusiform gyrus by the inferior temporal sulcus. The fusiform gyrus is separated from the
hippocampal gyrus by the collateral fissure. The anterior portion of the hippocampal gyrus
bends around the hippocampal fissure to form the uncus. The mesial surface largely the
hippocampal gyrus, slopes downwards to the inferior surface. Unit 2: Auditory perception,
Visual perception, Olfactory function

Auditory perception:

• Lesions in the auditory association cortex of the left side produce sensory aphasia. The
difficulty arises only when the patient has to distinguish speech sounds, and the older term
auditory or acoustic agnosia is in some way to be preferred since it points to the primary
difficulty from which a number of other symptoms flow. However, usage is too well
established to allow the change, and the term acoustic agnosia is best preserved for the
inability to distinguish non-verbal sounds, which is associated with right hemisphere lesions.
The disorder of phonemic hearing is produced largely by lesions affecting the superiority or
temporal gyrus in the region adjacent to the primary auditory cortex of the left hemisphere.
• A phoneme is the smallest distinctive group of sounds in a language, and one of the principal
tasks in learning a language is to distinguish readily between phonemes. The nature of
phonemic distinction becomes apparent when we endeavour to learn a new language since
the distinctive speech sounds are not the same in all languages and the difficulties which a
person may have with learning a new language will depend on the phonemic distinctions
which are brought to the task. The ease with which we are able to distinguish p and b in
words such as peach and beach will be lacking for people who primary language has but a
single phoneme for these sounds.
• It follows that a person who has acquired difficulty in discriminating between similar
phonemes will have difficulty in understanding spoken speech. Luria expresses this
concisely. As words in his own language fail to be differentiated his attitude towards words
in his native tongue will begin to resemble that to words in a foreign language (Luria, 1973).
• Since patient’s basic difficulty lies in auditory discrimination, they are deprived of the
regulation of their own speech via the monitoring of their own vocal productions. They are
therefore unaware of their own defective speech and hence see no necessity for correcting it.
Moreover, if they have difficulty in producing the correct word to name an object, prompting
is of no avail since they are unable to fit the information into a phonemic system. Structured
language disappears and is replaced by very fragmented utterances that have been called a
‘word salad’.
• The failure to distinguish the vital differences in the acoustic content of words means that
patients are unable to write material that is dictated to them whereas they maybe readily able
to copy verbal material when it is presented visually. An important exception to the inability
to write words from dictation is in the ability to write words which have become so familiar
that they no longer require precise analysis of their acoustic content. Prime examples of this
conversion of words into motor stereotypies are the patient’s signature and the most
frequently used words in the person’s trade or profession. Luria and colleagues have pointed
out that this is an example of how, the cerebral organization of a process may change over
time. In the process of learning to write words, careful acoustic analysis is needed at first, but
the motor aspects of writing become more and more automatic with use and less dependent
upon auditory discrimination so that the writing of a familiar word comes to have different
neuronal or cerebral organization from a relatively new word. This means that lesions in
different sites will have different effects and once again, a careful qualitative analysis of the
precise form of difficulty which the patient has (in this case with some words and not with
others) will point more precisely to the location of the lesion.
• As one moves away from the area surrounding the primary projection area for audition, there
is a decrease in phonological disturbances and in the region of the middle temporal gyrus, the
most prominent defect is one of audioverbal memory, an auditory amnesic aphasia. The
characteristic feature of these memory disorders is the inability to repeat a series of words
which has been presented acoustically despite the fact that the patient maybe able to retain
and repeat single words. With a series of words the patient may show a primacy effect (the
reproduction of the first word given) or a recency effect (the reproduction of the last word
 given), other members of the series being lost. Luria et al. (1967) have analysed these
disturbances of audioverbal memory and suggest that the fault lies in the increased mutual
inhibition of the auditory traces. This hypothesis is supported by their finding that increasing
the time interval between the presentation of members of the series greatly reduces or
eliminates three difficulty. The extension of the time between words is thought to reduce the
mutually inhibitory effects which adjacent members of the series have on each other. This
type of finding is also relevant to an examination of the amnesic syndrome which follows
bilateral mesial temporal lesions discussed below. The major pathology is in the dominant
hemisphere with the temporal lobe always affected.
• Left sided posterior temporal lesions sometimes leads to a difficulty in using words to name
objects, the so-called nominal aphasia. The patient perceived the objects and their
significance and can usually in a round-about fashion known as circumlocution, describe
their use of function. It is the association between the visual apprehension of a particular
object and a particular word is lost.
• A second effect of lesions which disrupts the coordinated action of vision and audition in this
borderland region between the temporal and occipital lobes is an inability to draw objects on
verbal request (‘please draw a clock’), while retaining the ability to draw the same object
when a copy is presented. It might be assumed that, because of disconnection of portions of
the auditory and visual regions from each other, words no longer evoke images which would
form the basis for executing a drawing of the object named.
• So far we have dealt with difficulties which arise with the understanding of speech sounds.
Man must also be able to discriminate and attach meaning to other environmental sounds.
The finding of impairment in the recognition of these non-verbal sounds was described in the
literature for many decades under the term acoustic agnosia, though this term is seldom used
in the recent literature, having been replaced by the term auditory agnosia. Even this term has
ambiguities. Used in a general sense it means that the person has a difficulty in recognizing
sounds both of speech and non-speech despite adequate hearing on audiometry. With the
increasing specificity of the neuropathology of causative lesions, it might be preferable to use
more category specific terms such as auditory sound agnosia, auditory verbal agnosia and
other categories in the way suggested by Bauer (1993).
• Unfortunately, the term auditory agnosia has been used somewhat indiscriminately at times to
refer to difficulty with the recognition of any kind of auditory material including speech. In a
review of the earlier clinical literature, Vignolo (1969) employed the term in its original sense
 of ‘defective recognition of non-verbal sounds and noises’. This group addressed itself to two
major aspects of the problem of auditory agnosia, namely the relationship of auditory agnosia
to aphasia and the relationship of auditory agnosia to hemispheric location of the lesion. The
two experiments described by Vignolo (1969) are summarized here in some detail. These
experiments have led to the concept of the double dissociation of auditory functions between
the two temporal lobes.
• In the first experiment, a test of auditory recognition was given to normal subjects and to
patients with unilateral temporal lesions (both right and left sided). Left-sided patients were
defined as aphasic if their scores fell below a certain point on one of a number of tests of
aphasia. These aphasic patients were then further categorized into types and degrees of
aphasia.
• The auditory test required subjects to select from four pictures on a card the source of a
common environmental sound played to them. The sounds were unambiguous, e.g., baby
crying, ambulance siren, yapping dog. Four separate pictures were presented with each
sound, each having the following categories of sound source: (i) the correct source (e.g.,
canary whistling); (ii) acoustically similar source (man whistling); (iii) similar class or
semantic category (cock crowing); (iv) unrelated sound source (train). Thus the subjects
could have three types of misrecognition, acoustic errors, semantic errors and unrelated
errors. The findings supported a distinction made by Vignolo between two different types of
auditory agnosia.
• An inability to discriminate accurately the sound pattern produced a perceptual discriminative
sound agnosia while an inability to associate the auditory stimulation with its meaning
resulted in an associative sound agnosia. As might have been anticipated, sound recognition
defects were more frequently associated with marked sensory aphasia than they were with
other types of aphasia. Thus auditory verbal comprehension and the recognition of non-verbal
sound sources were closely related.
• An examination of the type of error made by the aphasic and non-aphasic groups showed a
highly significant difference with regard to the ‘semantic’ errors but not to the other types,
i.e., ‘auditory’ or ‘unrelated’. This suggested that the difficulties in recognition by the aphasic
patients were due not to any inability to discriminate but to an inability to associate the sound
which had been perceived with its usual meaning. On the other hand, two patients with right
hemisphere lesions who performed poorly on this test did so because of an increase in
 acoustic errors, ie., their difficulty appeared to be discriminative rather than semantic
associative.
• The second experiment strengthened and clarified this distinction. Two tests were employed.
One, the Meaningful Sounds Identification Test, was similar to the test employed in the first
experiment, requiring the subject to select the correct pictorial representation of a well-known
environmental sound. The second task, the Meaningless Sounds Discrimination Test,
required the subject to discriminate between pairs of complex sounds which had been mixed
artificially in a sound studio. Again, groups of left and right brain damaged patients, and
normal subjects were used.
• Each of the brain damaged groups performed poorly on one of the tests, the deficit varying
according to the hemispheric locus of the lesion. Left sided damage was related to poor
performance on the test of semantic association (meaningful sounds), while this groups'
performance on the discrimination test (Meaningless sounds) was normal. The right
hemisphere group reversed the pattern of deficit, having a normal performance on the
semantic associative task and a very poor performance on the perceptual discriminative test.
In keeping with the findings from the first experiment, all patients with an exclusively
semantic-associative defect were aphasic.
• Though there have been very few cases of auditory sound agnosia described in the literature,
a case such as that of Fuji et al (1990) lends weight to the Vignolo findings. Their
righthanded patient could not recognize the meaning of non-verbal sounds while showing no
language comprehension difficulties and CT scan showed a lesion in the right temporal lobe.
• This double dissociation of function of the two temporal lobes with respect to auditory
perception further supports the lateral specialization of function shown by studies of material
specific memory defects, dichotic listening studies and cortical stimulation of the temporal
lobe regions, and the finding of asymmetry of auditory recognition is in keeping with the
broader notion of cerebral hemisphere asymmetry of function. These findings also have a
practical application in diagnosis, since they appear to make an unequivocal distinction
between unilateral lesions of the temporal lobes.

Dichotic listening studies

• Auditory material is presented simultaneously to the two ears by means of a stereophonic

tape-recorder. The material may consist of pairs of digits when three pairs of these are given
in fairly quick succession, the subjects can normally repeat all six digits usually reporting the
 three digits which were presented to one ear followed by the three presented to the other. In
right-handed subjects (assumed to be left hemisphere language dominant), the digits from the
right ear are normally reported first. This maybe assumed to be in keeping with the verbal
nature of the material and stronger contralateral representation of the auditory input
mentioned at the beginning of the chapter. This finding is sometimes referred to as ear
asymmetry or inter-aural rivalry. It might be better to avoid such terms since they focus
attention on the periphery rather than on the asymmetry of processing by the two
hemispheres.
• Attention was directed to this area by the early work of Kimura. In her studies with Milner at
Montreal (Milner, 1962), it had been found that certain aspects of the Seashore Measures of
Musical Talents differentiated between patients who had undergone right or left anterior
temporal lobectomy for epilepsy. In particular, right temporal lobectomy produced a marked
deficit of tonal memory while left-sided operations had no effect on this subtest. These
deficits appeared to be of a higher order or agnostic type, since hearing as measured by
audiometric testing was apparently unaltered. Following these findings, Kimura (1961)
employed Broadbent’s dichotic technique in the assessment of temporal lobe damage.
Patients with temporal lobe epilepsy were examined before and after lobectomy. Patients
with temporal lobe seizures performed poorly before operation, but following operation the
left temporal group became much worse while the level of performance of the right temporal
group remained the same. These right-sided patients, however, now reported more of the
right ear digits than they had before the operation. In more general terms, unilateral temporal
lobectomy impaired the recognition of material by the contralateral ear. The fact that the left
temporal lobectomy group performed very poorly is related to the verbal nature of the
material used, i.e., the deficit is a function of the type of material presented.
• Kimura (1961) assumed that the superiority of the right ear in dichotic listening (verbal)
experiments was due to a direct relation between cerebral dominance and the verbal nature of
the perception. However, subsequent studies have shown that much of the effect can be
accounted for by what has come to be termed the ‘ear order effect’ (Satz et al., 1965). This
effect represents a greater decay related to material reported from the ear which is reported
second. The first ear report is closer in time to immediate apprehension of the stimuli while
the second report is subject to decay in short-term memory.
• Clinical studies of amusia (inability to recall musical sounds) reported in the literature have
often given equivocal findings with respect to the question of localization. Though many
 cases implicate the anterior regions of the left hemisphere, some patients clearly have right
fronto-temporal lesions and jargon amusia accompanied jargon aphasia in one man with a left
parieto-occipital infarction. One rare case with a lesion in the right insular region showed a
receptive musical deficit which was dissociated from a normal appreciation of speech and
environmental sounds. Since amusia in its various forms almost always accompanies other
deficits, particularly aphasia, interpretation of test results has often confounded any loss of
musical recognition with verbal aspects of the test situation such as comprehension of verbal
instructions and naming difficulties.

Inferring language dominance

• It was hoped that dichotic listening results might be used to infer functional asymmetry of the
brain in individual cases without having to resort to the Wada technique of intracarotid
sodium amytal injection described below. Knowledge of cerebral dominance is desirable in
certain neurosurgical procedures and in the application of unilateral electroconvulsive
therapy (ECT). However, numerous studies attest to the fact that not only are the differences
small but the reliability of the usual method of dichotic recall is unacceptably low while
others question the dubious logic employed in drawing inferences from the test results.
• A more robust test named dichotic monitoring was developed by Geffen (1076). This
requires the subject to make a manual response on detection of the stimulus, rather than using
verbal recall. A validation study showed considerably larger measures of ear advantage than
for the usual dichotic recall method.
• While a more robust, non-invasive technique maybe helpful, even this fails to satisfy
requirements in one of the most common situations namely proposed temporal lobe surgery
for intractable epilepsy where amytal ablation remains the preferred technique.

Music and the brain

• Apart from dichotic listening studies, the testing of patients with unilateral lesions
(particularly in the temporal lobes) lends general support to a hemispheric asymmetry of
function with regard to the perception and execution of music, although the evidence as to
which particular characteristics relate to the dominant or non-dominant side is still far from
clear.
• Damasio and Damasio (1977) having reviewed the evidence to that time, felt the evidence
supported a major role for the right hemisphere for musical execution. As in the case report
 of McFarland and Fortin (1982), they felt that this could be dissociated from musical training
and experience.

The cocktail party effect

This term refers to the ability of normal individuals to note information, such as one’s name, in one
of a number of simultaneous auditory channels of information even though that particular channel
appears not to be the central focus of attention. This capacity is lowered on the contralateral side for
those who have undergone the operation of anterior temporal lobectomy.

Visual perception:

• The temporal lobes are neither concerned with the primary perception of visual information
nor with its elaboration into meaningful wholes. They are concerned however with the
integration of visual experience with all forms of sensory information coming from the
receptors of the other special senses and from the receptors of the bodily senses.

• Disturbances of all forms of perception of the individual’s internal and external words are
seen in all their complexity in temporal lobe epilepsy, the examples of which are described
below.
• The temporal lobes contain a portion of the optic radiations which curve forward into the lobe
after leaving the lateral geniculate bodies before looping back to their termination in the
occipital lobes. Temporal lobe lesions thus produce visual field defects which
characteristically affect the upper homonymous quadrants but may sometimes produce a
complete hemianopia. Even in cases, where no field loss was apparent to normal
examination, changes were detected after temporal lobectomy in the form of raised flicker
fusion thresholds for both left and right-sided cases. Differences between the impact of the
lesions according to the side have been demonstrated in a number of studies. Thus, Dorff et al
(1965) found that, using the method of presenting two stimuli simultaneously one to each of
the visual fields, the left temporal group was impaired in the right (contralateral) visual field,
while the right temporal lobe group was impaired in both left and right fields.
• Other studies have suggested that lesions of the right temporal lobe might produce
disruptions of visual perception that are not shown by comparable lesions on the left side.
Milner (1958) found that patients with right temporal lobe lesions had difficulty in
recognizing objects from an incomplete pictorial representation of them, a difficulty which
was not shown by patients with left-sided lesions. Kimura (1963) using tachistoscopic
 presentation found that lesions on the right-side impaired subject’s recognition when the
material was unfamiliar, while left temporal subjects were more impaired when familiar
material was being presented.

• Warrington and James (1967b) failed to confirm Kimura’s findings of impaired number
estimation on tachistoscopically presented material with right temporal damage, but did find
significantly raised recognition thresholds in the contralateral left visual fields. Rubino
(1970) found that right temporal lobe removal rendered the patient less able to identify
meaningless visual patterns than did left-sided removals.
• Further support to the association of special defects with right temporal lesions was given by
Lansdell (1962a) who found right temporal lobectomy patients to be poorer on a design
preference test than left lobectomy patients. In a later study, Lansdell (1968) reported that
right-sided operations led to poorer performance also on a visuospatial abstract reasoning
task that was relatively unaffected by left-sided operations. The more extensive the removals
were on the right side, the greater were these deficits.
• Though both temporal lobes are intimately concerned with perceptual processing, it appears
that the lateral specialization shown in auditory perception extends also the visual modality in
this area.

Olfactory function

• The olfactory receptive area is located in the uncus and adjoining parts of the
parahippocampal gyrus.
• Damage to the olfactory pathways or cortex produces anosmia. Olfactory hallucinations are
often an indicative of the lesions in the region and are known as uncinate fits sometimes
occurring as an epileptic aura.
• Following temporal lobectomy, Rausch and Serafetinides described an elevation in the
threshold of detection for the quality or identity of an odour.
• Lobectomy patients showed more error in odour recall (right lobectomy patients performing
more poorly than left).
• Another study showed that right lobectomy patients were only impaired on matching odours
but they were able to discriminate the odours.
• Esknazi et al(1983) found no laterality effect though subjects post operatively had poorer
immediate and delayed odour memory than controls and also showed impairment on wide
range of tests of olfactory functioning.
Unit 3: Behavioural changes with temporal lobe epilepsy

• Several studies have shown that there was a higher frequency of psychopathology in those with
temporal lobe epilepsy than when compared with other neurological disorders.
• Bear and Fedio(1977) compared patients with unilateral temporal lobe foci with normal subjects
and those with neuromuscular disorders on specific aspects of behaviour.
• Hermann and Riel (1981) supported the findings of Bear and Fedio’s point by finding
characteristic differences between those with temporal lobe seizures and those with generalized
epilepsy on self-report questionnaire. It was found that the defects were in desire for social
affiliation, circumstantiality, religious and philosophical interests and deepened affects were
seen among temporal lobe epileptics.
• Bear theorized that the connections between sensory cortex and limbic system form the
substate for ‘attributing visceral or emotional significance to perceived stimuli. This could
lead to an increased connection as the result of heightened electrical activity in temporal lobe
seizures.
• Flor and Henry(1969) did an evaluation on 50 temporal lobe patients who had psychotic episodes
and he claimed that temporal lobe epilepsy of the dominant hemisphere(left) predisposed to
psychosis and it tended to be schizophreniform in nature and psychosis accompanying non-
dominant hemisphere(right) epilepsy tended to be manic-depressive in nature.
• Lennox introduced the term ‘Temporal Lobe Epilepsy’- which are complex partial seizures
arising in thee temporal region.
• The complexity of the syndrome lies in the spread of excitations from several regions but the
mesial temporal areas are more often involved than other areas.

Unit 4: Hallucination and illusions

• If the perception refers to a person or object present in the environment we speak of an illusion or
false perception. With temporal lobe attacks part or all of the object may be distorted, eg.
everything may appear visually larger or smaller (macropsia, micropsia) or the relative size of
parts may appear distorted (metamorphopsia), or sounds louder or softer than usual.
• These distortions are often accompanied by a feeling that the person is somehow detached from
his/her own body (depersonalization) or that things are unreal (derealization).
Hallucinations refer to perceptual experiences which do not correspond in any way to stimuli in
the current environment.
• The hallucination may be accompanied by emotional experiences which are usually unpleasant,
though pleasurable feelings and even short periods of ecstasy have been described infrequently.
Williams (1956) has examined these emotional experiences and related them to specific locations
in the temporal lobe.
The change in affect at the time of seizure is most commonly that of fear (Williams 1956,
Daly 1975), and this fear has been evoked by stimulation through implanted electrodes (Bancaud
et al., 1994). Some patients experience unprecipitated fear in the interictal period (Hermann &
Chhabra 1980).

• For learning to take place, or to decide whether an object or situation is ‘familiar’, one must
compare the present sensory input with the neural record of past experience, and he interprets the
data of temporal lobe epilepsy as demonstrating the presence in the temporal lobe of
‘coincidence detection circuits’. If the comparison of the input with the record of the past
produces a coincidence or familiarity response, the present stimulation will appear familiar even
if nothing similar had occurred in the subject’s prior experience.
• This is the well known phenomenon of déjà vu (seen before), which is experienced at times by
normal subjects but more frequently and with greater vividness by some patients with temporal
lobe epilepsy. On the other hand, a matching which produces a ‘no coincidence’ response leads
to the experience of ‘jamais vu’ or ‘jamais entendu’ (never seen, never heard), eventhough the
stimulus pattern or one very similar has been frequently encountered in the past.

• Penfield (1954) referred to these alterations in the perception of the present as ‘interpretive
illusions’. While déjà vu experiences are associated with pleasant or neutral affect, those of
jamais vu may be accompanied by negative affect such as fear (Sengoku et al 1997) .

Electrical Stimulation (Unit 5)

• Temporal lobe surgery, particularly surgical procedures for the treatment of intractable complex
partial seizures, presented an opportunity to study the effects of electrical stimulation from both
the temporal cortex and subcortical regions. Several classes of phenomena have been elicited :

I. visceral sensation, fear and anxiety

II. complex hallucinations and experiential changes including déjà vu.
iii) Changes in memory.
•
• Only a small proportion of stimulations produce mental phenomena, even where evoked
potentials and/or after discharges have been recorded certainly on less than 10% of occasions.
In 1938, Penfield produced for the first time an evocation of experience by stimulating the
temporal cortex in a conscious human subject.
These experiences were of two kinds: firstly the evocation of an experience which the subject
had undergone on a number of previous occasions during epileptic seizures, and, secondly,
the production of previous happenings which had not been seen during the attacks but here
also clearly related to specific prior experiences. Penfield used the term experiential
hallucination when the phenomenon occurred spontaneously and experiential response when
it was elicited by stimulation.
• Several features stand out clearly in the very large number of cases where stimulation of the
brain was employed (Penfield & Perot 1963) :
i) Despite the stimulation of practically every accessible spot on the cerebral cortex,
experiential responses were evoked only from the temporal lobe (a total of 612
patients in Penfield’s series were stimulated in non-temporal areas and produced not a
single experiential response).
ii) In almost all cases of evoked experiences, the patients were suffering from temporal
lobe epilepsy.
iii) Only about 8% of temporal lobe cases stimulated gave rise to experiential responses.
iv) Responses were evoked from both sides of the brain but there was a marked
asymmetry.
• The greatest concentration of experiential responses (on electrical stimulation) was in the
superior temporal convolution of both hemispheres, with the frequency on the right side
being greater than that on the left. On the right side, too, the points giving rise to experiential
responses extended more posteriorly along the superior temporal convolution and the
posterior portion of the whole right lobe is productive, while the corresponding regions on the
left are almost silent to stimulation.
• Auditory responses evoked by stimulation are concentrated on the superior regions of the
lobes with greater frequency on the right. The responses were most often a voice or voices,
though sometimes meaningful environmental sounds or music were elicited. Combined
 •
auditory and visual responses were evoked on only very few occasions. Experiences of music
were more frequently reported from the right side.
• Temporal lobe stimulation sometimes evoked visual experiences. There was an even greater
preponderance of responses from the right side and the experiences were evoked over a much
wider range of points in the right temporal lobe than was the case with auditory responses.
• Finally, there is a small amount of evidence that gelastic seizures (those characterized by
outbursts of laughter) are dependent upon pathology in the temporal lobes.

Unit 5- Lesion and Cognitive Change

1. Unilateral lesions
Intelligence test differences
• There have been frequent assertions that the differential effects of unilateral temporal lobectomy
according to the side of the lesion are examples of what Blakemore (1969) calls ‘the broad
generalization that lesions occurring in the hemisphere of the brain which is dominant for speech
produce deficits in performance on tasks which are essentially verbal in nature, while lesions in
the non-dominant hemisphere produce performance impairments on essentially nonverbal
(visualspatial and perceptual-motor) tasks’.
• Left unilateral anterior temporal lobectomy is often followed by aphasia which is, however,
transient in nature. Though language disturbance may cease to be clinically apparent, there are
numerous studies which show that there are verbal deficits which are apparent for some time after
operation when appropriate tests are employed.
• The early studies of Meyer and Yates (1955) and Meyer and Jones (1957) suggested that the
decline in verbal intelligence test scores which was found by studies such as Milner and Meyer
(1959) after left temporal lobectomy was an aggravation of a deficit which patients with a left sided
lesion had before operation.
• The three latter studies reported no significant change in the verbal subtests of the Weschler Scale
after operation on the right temporal lobe. Milner’s data suggests that there was also no decline on
the Weschler Performance subtests with right sided operations.
• However Milner (1979) reinterpreted Milner’s data in the light of the large practice effect from
Form I to Form II of the Weschler –Bellevue Scale which has been demonstrated by Gerboth
(1950), and came to the conclusion that the data did in fact demonstrate a decline in Performance
 •
Scale score after right temporal operations. Care must be taken in allowing for practice effects in
neurological populations since many such groups fail to show such an effect (Shatz 1981).
• Blakemore and Falconer (1967) also described a lowering of the Performance Intelligence Quotient
after right temporal lobectomy, Hebb (1939) had noted a lowering of non-language abilities,
particularly those associated with visual form perception.
• The general relation of verbal deficits with left and performance deficits with right sided-lesions
was supported by the studies of Lansdell, Dennerll and Blakemore (1966). On the other hand the
verbal versus non-verbal character of deficits related to the laterality of the lesion was not
confirmed by Parsons and Kemp (1960) .
A rather different set of findings was reported by Halstead (1958) on 21 cases with epilepsy
subjected to mall anterior temporal lobe resections. Firstly, the results of the operation on the left
versus the right side did not support the contention that important differences exist between the
dominant ad non-dominant temporal lobes.
• Secondly, of major interest was the fact that a significant difference patients and control subjects
which existed on several intellectual measures before operation actually improved, though
impairment in relation to normal performance was still seen on some tasks. Such results may be
due to the smaller nature of the operations compared with the larger temporal excisions in Milner’s
series and possible differences in the patient population selected for operation.
• Several follow-up studies after anterior temporal lobectomy have shown recovery of both the
verbal losses which follow left-sided operations and the non-verbal losses which follow right sided
ones within a year of surgery. The latter study covers a period of 10 years and it is difficult y
reconcile this with the findings of Meier and French (1966) that, while performance scale scores 1
year after right-sided operations showed no decline over the preoperative level, the scores at 3
years did show such a decline.
Material Specific Memory Loss

• The introduction of anterior temporal lobectomy as a standard treatment got intractable complex
partial seizures offered a unique opportunity for the study of the role of the mesial temporal
structures in memory processes. The resection typically includes the anterior 6 cm or so of the
temporal lobe and the underlying structures, the uncus, amygdaloid nucleus, and part of the
hippocampus and parahippocampal gyrus.
 •
• The evidence from this source strongly supports a complementary role for each temporal lobe.
Many studies have reported loss n verbal memory tests postoperatively, although this loss is
seldom of clinical significance, i.e. it does not inconvenience the patient in everyday life.
• Support for this relationship comes from other unilateral lesion studies, for example Coughlan and
Warrington (1978) found patients with left hemisphere lesions to be generally poorer than those
with right hemisphere lesions. Of the left sided group, those with temporal lesions were impaired
than those with lesions elsewhere in the hemisphere.
• Operations and lesions of the right temporal lobe differentially affect visuospatial and non-verbal
pictorial material. Smith and Milner (1981) also implicated the right temporal lobe in the memory
for the location of objects.
This evidence is usually interpreted as showing a material specificity of memory loss with
unilateral temporal lesions. Some evidence for the opposite point of view came from Meyer
(1959).
• Following an earlier study which demonstrated a severe impairment with aurally presented
material for left sided lesions and not right, Meyer specifically tested this proposition employing
both visual and auditory (and tactile) modalities in an examination of learning with both verbal
and non-verbal materials.
• Right sided removals produced no postoperative deficit, while left-sided removals produced a
marked impairment of verbal paired associate learning only with auditory presentation and not
with visual. Several other studies support the view that, while patients with left temporal or left
temporoparietal lesions may have difficulty with verbal memory for all forms of auditory
materialwords, letters, numbers-they have little or no difficulty with the same material versus
modality specificity still remains to be resolved.
• There is no doubt of the greater sensitivity of verbal memory to left sided lesions, the deficit being
demonstrable with cerebral tumours and after unilateral ECT on the left side but not on the right.
• Finally, Blakemore (1969) reported an earlier study in which the rate of presentation of items was
varied by altering the time between words and between pairs in paired associate learning. Patients
after left temporal lobectomy showed the anticipated deficits at normal and rapid rates of
presentation but, when the rate was slowed appreciably, the patients demonstrated that they could
learn almost as well as before operation.
 •
• Blakemore argued that the longer time intervals allow verbal mediation to be effective. Thus the
frequent assertion that the deficits after operation should be interpreted as learning deficits as such
should be viewed with caution.
• But many comparisons of temporal versus other epileptic groups and with normal have failed to
find a significant difference on memory tests. Others, while finding memory impairment in the
temporal lobe patients, found no relationship between laterality and specificity of memory loss.
• Unilateral electroconvulsive therapy has provided further evidence for lateral specificity of
memory function. Inglis (1970) pointed to a close resemblance between the effects of temporal
lobectomy but not by right while right sided ECT has a differential effect on non-verbal memory.
• The demonstration of detrimental effects of bilateral ECT or dominant side ECT, combined with
the demonstration of comparable effectiveness of non-dominant ECT led to fall off in the use of
the first two procedures.
Semantic Memory Loss
The first clear description of neuropsychological findings in three relatively several of the y pure
cases of semantic amnesia was given by Warrington (1975). A small number of cases of selective
impairment of semantic memory have appeared since, though some have had accompanying
defects.
• Several of the reports have concerned patients with widespread disorders such as encephalitis but,
even here, the critical neuropathology appears to be in the left mesial temporal region. In one such
case with encephalitis and a striking dissociation of semantic from episodic (autobiographical
memory), MRI showed damage almost completely confined to the left mesial temporal region.
• A semantic memory disorder appears as the principal feature of the recently described semantic
dementia. This is clinically a grammatically fluent form of progressive aphasia, in which
progressive dissolution of the lexicon occurs as a prominent feature, with preservation of nonverbal
new learning, orientation and aspects of everyday memory allowing differentiation from typical
Alzheimer’s disease
• Pronounced dominant inferior temporal lobe atrophy is seen on neuroimaging studies, though
asymmetrical bilateral lesions are also seen.

Amytal Ablation- The Wada Technique

• In 1949, Wada developed the method for determining directly the side of the hemisphere
which played the major role in subserving speech functions. A temporary cessation or
•
functional ablation was affected by injecting a solution of rapidly acting anaesthetic agent
into the internal carotid artery which supplies one side of the brain.
• This intracarotid sodium amytal injection technique produced the following evidence of
functional loss: (i) hemiplegia; (ii) hemianaesthesia; (iii) half visual field loss
(hemianopia)-all on the side opposite the side of the injection.
• If the hemisphere injected was dominant for language, aphasia was also produced. These
effects within about 5 minutes, though subtle changes in function could be elicited on
careful examination for as long as 30 minutes after the injection.
• Even where amytal injection produces marked difficulty of expressive speech, some
patients may preserve the ability to comprehend semantic relations.
• In a recent study with a large number of subjects, Wyllie et al (1990) confirmed previous
findings that subjects with left hemisphere language dominance shown on Wada technique
revealed no language areas in the right hemisphere on cortical stimulation, but two of those
with right hemisphere dominance had language areas on the left.
The amytal ablation technique has several advantages. Firstly, it allows the neurosurgeon to
determine the lateralization of language function quote unequivocally, and to gauge the probable
effect on language of an operation in either hemisphere, since separate injections can be made on
either side on two different occasions.
• The method came to be used extensively to determine the anticipated effects on memory
as well as language, most frequently in anterior temporal lobectomy for the treatment of
epilepsy. Verbal and non-verbal tasks were administered during the period of functional
ablation.
• The rationale for Milner’s test of memory and similar procedures was based on an
assumption derived from the findings stated above, namely that the loss of function in only
one temporal lobe does not produce a generalized memory loss. However, if an
unsuspected lesion is affecting the hippocampal area of the opposite hemisphere, then
amytal ablation of on temporal lobe should produce transiently the functional effect of a
bilateral lesion, namely, the pervasive non-specific amnesic syndrome
• This generalized memory loss should be apparent for the time of the ablation and clear up
as he effect of the anaesthetic agent wears off. This reversible functional ablation greatly
diminishes the potential risk of producing a lasting undesirable result from surgery.
•
• Left/right asymmetry of performances seems reliable I predicting amnesia risk for those
with leftsided lesions, but not for those with right-sided lesions, and even the type of
stimulus material used has a bearing, e.g. presentation of real objects appears more reliable
than the much more commonly used line drawings.
• The carotid amytal ablation technique has also been used in testing for lateralization of
functions other than language. Bogen and Gordon (1971) looked at musical ability during
depression of activity of the non-dominant (right) hemisphere in six patients.
• Injection of the right sided caused a marked temporary disturbance of singing ability,
whereas in five of the six patients ‘speech remained unaffected except for slight slowing
and slurring of words and the presence of some monotonicity, the intelligibility and
rhythmicity of speech were hardly affected’. Such a finding is in keeping with the evidence
cited elsewhere of the greater importance of the non-dominant hemisphere in certain
musical abilities.
• On the negative side Serafetinides (1966) noted that visual recognition of geometrical
designs was not affected by ablation of either side though the impairment of verbal recall
with amytal ablation of the dominant hemisphere was again confirmed. The most
important contribution to everyday uses of amytal ablation technique is that of Bouwer et
al (1993).
Finally, of great clinical importance is the basing of predictions of postoperative functions,
particularly of memory, on ‘passing’ or ‘failing’ tests such as the intracarotid amytal test. This is
of great importance where left-sided resection is being proposed since some studies indicate that
reports from both patients and families demonstrate significantly poorer memory outcome in those
patients who have shown no evidence of hippocampal sclerosis preoperatively than in those with
evidence of sclerosis.
 2. Bilateral lesions and general amnesic syndrome
• Unlike the mild material specific memory losses caused by unilateral lesions, the memory
difficulties of patients with bilateral lesions affecting the medial temporal regions are profound,
pervasive and generally lasting. Several lines of evidence support this relationship.
• The first strong line of evidence came from a prolonged and intensive series of investigations
carried out on a patient with bilateral operations that included an extensive amount of medial
temporal tissue including the amygdala and much of the hippocampi. This patient, known as HM,
developed a stable amnesia almost completely free of other cognitive deficits which has persisted
for decades.
• Immediately after the operation, HM demonstrated both retrograde and anterograde amnesia. The
retrograde amnesia cleared but the patient was left with some confusion about the chronological
order of events, particularly with regard to a period of 1-2 years before the operation.
• On the other hand, the anterograde amnesia remained severe with an almost total lack of
registration for everyday events. He repeatedly re-read the same papers and repeated tasks over
and over without giving any evidence of having done them before.
• He failed to learn the location of his house or the location of objects within it. He was unable to
learn the name of visitors even after they had been visiting the house frequently over some years
and failed to recognize them.
• On the other hand, HM’s immediate span of attention was normal. He could, provided there was
no distraction, repeat a normal span of six or seven digits.
• This preservation of immediate memory in the clinical testing of amnesic patients had been
reported many years earlier by Zangwill (1946) and has been confirmed frequently since. The
preservation of intelligence can be seen in a reported Weschler Intelligence Quotient of 118 some
9 years after operation.
The marked degree of anterograde amnesia in this syndrome has led to a tendency on the part of
some writers to overgeneralize and oversimplify the learning and retention deficit. There is now
ample evidence that some learning and retention takes place even in such pronounced cases as HM.
• Motor skills do not suffer to the degree shown by many other tasks. HM improved his performance
with practice on a mirror drawing task even though he was unaware on a number of manual
tracking and coordination tasks.
•

• In 1962, Milner reported HM’s complete inability learn a visually guided stylus maze. However,
when the number of choice points was reduced so that it fell within his immediate memory span,
he demonstrated extensive saving in the number of trials to relearn the maze a week after the initial
trials and a comparatively rapid relearning after 2 years.
• The learning occurred despite HM’s denial of previous experience with the tasks. Milner (1970)
also demonstrated that HM was able to learn the identity of 20 incomplete outline drawings.
• When retested 1 hour after first exposure to the stimuli he showed even greater savings than a
group of normal subjects.
• Amnesic syndrome with unilateral lesions-Despite the weight of evidence in favour of the
amnesic syndrome being produced only by bilateral lesions, occasional reports suggest that
unilateral lesions may produce the loss. Penfield and Milner (1958) reported two cases of the
syndrome after unilateral anterior temporal lobectomy in the hemisphere dominant for language.
• They attributed this effect to the unsuspected presence of a lesion in the hippocampal areas of the
non-operated side so that was a unilateral operation in one sense produced a bilateral lesion in
another.
• Their contention was borne out in one case by the finding of a wasted hippocampal region in the
non-operated hemisphere at post-modern and by the presumptive evidence of EEG abnormality in
the other case in the side opposite the operation.
• In 1964, Dimsdale and colleagues described the occurrence of a case of general amnesia after right
lobectomy in which there had been no neurological evidence whatsoever of a lesion which was
tem compounded with the right-sided lobectomy to produce the effect. This implied bilaterally of
lesion, one preoperative (left hippocampal) and one operative (right temporal lobectomy), was
confirmed 8 years later by the finding of left hippocampus in the ‘silent’ temporal lobe may be
completely wasted.
Our patient, who was a candidate in prospect for left-sided lobectomy, died of a cause unrelated
to her epilepsy and the right hemisphere at autopsy showed wasting in the hippocampal region.
Earlier psychological testing in this case had pointed to a lowering of function in certain non-
verbal functions usually associated with the ‘minor’ hemisphere. Cases of this type are now being
shown clearly with MRI scanning.
• Finally the cases described by Stepien and Sierpinski (1964) are quite contrary to all the above
findings. These three cases had a general memory defect before the operation and EEG evidence
of abnormality on both sides.
•

• Removal of one temporal lobe, rather than aggravating the defect as Milner’s hypothesis would
suggest, resulted in a disappearance of the amnesia. These findings are difficult to reconcile with
the numerous studies cited earlier.
• A further landmark in the neurological basis of memory disorders was the report of Rose and
Symonds (1960) of four cases of severe amnesia occurring after recovery from what may be
assumed to be viral encephalitis. Barbizet (1960) pointed to a similar post-encephalitic case
reported by Hillemand and colleagues in 1931, a patient whose memory disorder was essentially
unchanged when Barbizet examined him 36 years later.
• The features of this case are essentially those of the general amnesic syndrome, namely, gross
defect of recent memory with difficulty in the registration of on-going events and some retrograde
amnesia, with relatively little impairment of other intellectual functions. A constant feature of these
hippocampal amnesias is preservation of immediate memory.
• The most common cause appears to be a necrotic encephalitis of herpetic origin causing bilateral
hippocampal damage and sometimes other lesions. The neuropathological evidence reviewed is
some detail in Brierley.
• The third major cause of medial temporal amnesia is bilateral compromise of the posterior cerebral
arteries. The memory problems may be of a transient or permanent nature.
• Occasionally, after several transient amnesic episodes the patient may die of a massive
cerebrovascular accident typically, the onset is sudden so that the term ‘amnesic stroke’ is used.
Bilateral infarction is common, since both posterior cerebral arteries arise from the single parent
vessel, the basilar artery.
• For the same reason patients often suffer a cerebral blindness; which may partially remit.
• Post-mortem examination of the brain showed old bilateral infarctions in the inferomedial portions
of the temporal lobes. Since that time further studies have described the association of serious
memory defect in life with post-mortem evidence of bilateral infarction in the territory of the
posterior cerebral arteries.
• Trillet et al (1980) described 30 cases of vascular origin (17 permanent and 13 transient) with
anterograde amnesia as the principal finding. All had cerebral blindness at the outset. Benson et al
(1974) reported the acute onset of amnesia in 10 patients, associated with unilateral or bilateral
visual field defects clearly caused by posterior cerebral artery territory infarction.
• A severe amnesia may be produced by infarction n the dominant hemisphere only. It is possible
that, because of the common use of largely verbal or verbally mediated tests of memory, these
•

cases may appear to be instances of the general amnesic syndrome rather than, as we believe, cases
of severe verbal specific memory deficits.
• It is also possible that there may be bilateral but asymmetrical involvement of the posterior regions
with pathological emphasis on the dominant side. One again, modern imaging techniques do much
to clarify the nature and extent of the lesions.
• Whatever turns out to be the case, severe memory impairment is related to the dominant than the
non-dominant hemisphere in unilateral cases. The predominant mechanism of production of
amnesia may be involvement of the inferomedial parts of the temporal lobes, including the
hippocampus.
• The single case of a severe amnesic syndrome produced by infarction in the area of the left anterior
choroidal artery would fit this proposition. Finally, hypoxic injury to the brain following cardiac
or respiratory arrest may result in cognitive loss, in which prominent feature is a general amnesic
syndrome.

Preserved learning in amnesia

• Not all memory functions are lost even in a ‘general’ amnesia; for example it has been known since
the work of Corkin (1965,1968) that motor learning may be intact in the amnesia which follows
some bitemporal lesions. Other tasks without a significant motor component may also be
preserved, and much attention has been given to the topic, often termed procedural or implicit
memory, in recent years .

Transient Global Amnesia (TGA)

• This term was introduced by Fisher and Adams for a condition described earlier by Render and
Guyotat and Courjon. The attack is marked by confusion, during which the patient has repetitive
queries and shows a total inability to form any new memories.
• There is retrograde amnesia during the attack for events extending back for days or, in some cases
even years before the present but, as the confusion and anterograde amnesia clear, the retrograde
amnesia shrinks to leave amnesia only for the period of the attack itself. Most often there are no
accompanying neurological signs and symptoms.
• The condition, unlike transient ischemic attacks, is benign with no added risk of stroke or other
vascular problems shown on follow-up. TGA is more often seen in those of middle age or older.
•

• The event lasts around 4-8 ours, may be a single event, or may recur a number of times. There is a
strong association of TGA with migraine, with dozens of reports of transient amnesic attacks in
migraineurs.
• The amnesic episodes appear to be precipitated by factors which are commonly factors which are
commonly inducers of classical migraine. In one migraineuse the amnesia was largely verbal, the
non-verbal memory being relatively spared.
• Matias-Guiu and Codine (1986) reported a further four cases of transient partial amnesia
restricted to verbal material and listed features which should be present for a case to fit this
category. This is important since there are numerous cases of transient memory difficulty which
do not meet the criteria for either transient global or partial amnesia.
• Despite a strong association with migraine, TGA has been reported with a particularly wide range
of factors, such as immersion in cold or hot water, sexual intercourse, or highly emotional
experience, and the disorder has also been described with a wide variety of pathological conditions
such as mild head trauma, hydrocephalus and cerebral tumours. There are several reports of TGA
after vertebral angiography.
• The most common opinion is that the condition is due to temporary ischemia in the territory of the
posterior cerebral arteries, though a pioneer in the field was strongly opposed to this view.
• Neuropsychological testing: With the growing interest in TGA, especially as a tool for the
understanding of the neural basis of memory functions, there have been a number of reports of test
findings both during and after the attacks. One study of two patients has shown the preservation
both of semantic memory and frontal executive function assessed by several measures. In essence
the transient condition closely resembles the lasting general amnesic syndrome of central origin
following bilateral damage to the medial temporal or medial diencephalic regions. For this reason,
the present authors would prefer the term transient general amnesia for the condition since, unlike
the global amnesia of late Alzheimer’s disease, immediate and long term memory are usually
much less affected.
• The principal features are:
Preservation of immediate memory.
A general, i.e. verbal and non-verbal, anterograde amnesia. On occasions we have noted
cases where non-vernal memory does not appear to be as severely affected as verbal
memory.
•

Some retrograde amnesia. Following the attack this retrograde amnesia shrinks rapidly,
though many are left with a hiatus of a day or two. Pure retrograde amnesia is also
reported.
Other cognitive functions such as language, problem solving and visuospatial functions
are well preserved. Studies report poor copying of a complex figure and possible
difficulty with confrontation naming. Preservation of other functions stands I stark
contrast to the amnesia.
• Recovery and Prognosis. From follow up studies, it is apparent that recovery is more gradual
than is suggested by the clinical picture, and a residual memory weakness in the form of a new
learning deficit may be present in some cases even months after the attack. Minor deficits may
follow multiple attacks, but these may not be of clinical significance.
• One case whose memory had been tested before the attack showed no deficit following the
episode, even showing a normal ‘practice effect’ on one of the tasks. Few data exist on other
psychological functions in TGA patients.
• Mazzucchi and colleagues (19800 appear to show some cases with a lower verbal than performance
IQ, differences in this direction being unusual in subjects of normal or above normal ability. Other
studies have not shown this trend consistently and it would be wrong to conclude that there is a
hemisphere relationship; certainly there are cases of TGA with right hemisphere lesions.
Baron and co-workers suggest that, like permanent amnesia, TGA maybe a core
syndrome with several possible foci of dysfunction along the neuronal networks that
subserve explicit memory
Whiplash Amnesia
• A type of transient amnesia with all the features of an episode of transient global amnesia
following whiplash injury to the neck, usually in motor vehicle accidents. Apparently full
recovery maybe a function of the type of evaluation since the detailed examination of a
case described by Walsh revealed major recovery but significant residual memory
difficulty.

Vertebrobasilar Insufficiency (VBI)

• Atherosclerosis in the posterior circulation, viz, verbal, basilar and posterior cerebral arteries,
may cause transient attacks of brain stem vascular insufficiency, the most common symptoms
being those of vestibular and cerebellar disturbance. Studies show that patients showing
•

symptoms of VBI are often forgetful have poor concentration and occasionally have attacks of
TGA. Two small studies suggested that patients with chronic VBI may show evidence of a mild
memory problem which has the characteristic of an axial amnesia. Amygdala Lesions and
Cognition
• Patients with isolated lesions of the amygdala are rare. The cases available support a role in
emotional processing, including the linking of sensory information to emotionally relevant
behaviours, particularly responses to fear and anger,
• A comparison of the ability to acquire either visual or aural conditioning responses to aural
unconditioned stimuli showed a double dissociation between lesions involving the amygdalar
and hippocampal regions. The amygdala was implicated in conditioned learning but not
episodic memory.
• Normal autonomic responses to the unpleasant unconditioned stimulus occurred even when an
amygdala lesion was present. Bechara et al (1995) conclude that thee amygdala is essential for
the coupling of sensory stimuli with affect-the establishment of sensory-affective association,
regardless of modality of presentation, and that the amygdala is essential for association of
contextual (complex) or discrete (simple0 cues with affect, whereas the hippocampus is critical
for learning the relations among contextual cues.
• Contralateral startle response, recognition of facial emotion and other aspects of facial gesture
were reported as deficient with a unilateral right amygdala lesion, leading to the hypothesis that
the learning of socially relevant stimuli may be in part mediated through the amygdala.
 MODULE 5 – PARIETAL LOBE

Unit 1: Basic Anatomy of Parietal Lobe

• The parietal lobe has two main surfaces: lateral and medial surfaces.
• The anterior border of the lateral aspect is formed by the central sulcus, while the posterior
border is formed by the parieto-occipital sulcus and a line drawn from the end of this sulcus
to the pre-occipital notch on the inferolateral border of the hemisphere.
• The lower border separates the inferior part of the parietal lobe from the superior portion of
the temporal lobe. It is made up of the lateral sulcus and a line continued back from it to
reach the posterior line of demarcation.
• Two well marked sulci lie within the parietal lobe. The post central sulcus delimits the
postcentral gyrus which is concerned with somatic sensation. The intraparietal sulcus runs
roughly parallel to the lower margin of the lobe about midway in the lobe, separating it into a
superior and an inferior parietal lobule.
• The anterior of these is the posterior branch of the lateral sulcus, while just posterior to it is
the end of the superior temporal sulcus. The cortex of the inferior parietal lobule around the
end of the lateral sulcus is the supramarginal gyrus that around the superior temporal sulcus is
the angular gyrus. Not infrequently the sulci of the supramarginal and angular gyri are
independent of the lateral and superior temporal gyri.
• The anterior border of the medial aspect of the parietal lobe is formed by a line which extends
about midway through the paracentral lobule from the point on, the superomedial border of
the hemisphere reached by the central sulcus to the top of the corpus callosum.
• Thus only the posterior half of the paracentral lobule belongs to the parietal lobe. The
posterior border is formed by the parieto-occipital sulcus, which is usually very distinct. The
region anterior to this sulcus is the precuneus which extends anteriorly to the continuation of
the post-central gyrus and is continuous around the subparietal sulcus with the cingulate
gyrus. A branch or continuation of the cingulate sulcus, the marginal sulcus separates the
anterior border of the precuneus from the posterior border of the paracentral lobule.
• Following this description of the parietal lobe, as an empirical convention it would be wise to
repeat the warning of Critchley (1953) who quoted that parietal lobe cannot be regarded as an
autonomous anatomical entity; its boundaries cannot be drawn with any precision except by
adopting conventional and artificial landmarks and frontiers. Rather, the area can be
 described in a less narrow terminology, and one which would include the whole retrorolandic
complex, or a three-dimensional temporo-occipital territory as a functional domain.

Unit 2: Sensory and perceptual disturbances –

• The primary reception area for the numerous forms of somatic sensation has its principal locus
in the post central gyrus. The secondary or association cortex posterior to this is thought to
deal with the elaboration of the discrete elements into meaningful wholes so that disorders
with damage away from the somaesthetic area tends to be more complex, i.e., tend to be
perceptual or cognitive in nature rather than simple disturbances of sensation. Further, afield
in the region of the ‘temporo-parieto-occipital crossroads’, the disturbances tend to be those
which reflect a disruption of intersensory or cross modal association and integration.

Following this ‘anatomical’ division of deficits according to type of cortex, three types of deficit
have been selected for treatment:

(1) Somatosensory discrimination

(2) Disorders of tactile perception
(3) Disorders of intersensory association

Somatosensory discrimination:

• Somatic sensation is made up of a number of separate modalities. Among these are touch,
pain, temperature, body position, sense, kinaesthesis and vibration. The detailed organization
of each of these is dealt with in texts of physiological psychology.
• The somatosensory system is able to combine information from different modalities in
different locations and with different temporal relationships.
• The earliest neuropsychological studies of the cerebral basis of somatic sensations in man
came from the examination of missile wounds to the head. The value of these studies suffered
from the inability to localize accurately the site of the lesion for correlation with the results of
their painstaking examination of sensory-perceptual capacities.
• The introduction and increasing use of cortical ablation for the removal of cerebral scars
allowed Penfield’s group to make the first serious examination of sensory defects following
circumscribed ablation of cortical tissue. Employing the detailed examination procedure
outlined by Head, Evans examined 17 cases, nine posterior and eight anterior.
• Evans concluded from the examination of these cases that damage to the extraparietal areas,
the pre and post-central gyri and the central portion of the parietal lobe led to transient
sensory dysfunction, if any, ‘while limited excisions in the region of the supramarginal gyrus
caused extensive and permanent loss of somesthetic sensation’.
• In 1956, Hecaen et al, in a study of patients with surgical lesions of the minor hemisphere
reported that patients with parietal lesions showed no increase in sensory thresholds unless
there was also involvement of the rolandic region. They also noted difficulties with complex
sensorimotor tasks such as using scissors, dressing and making block constructions, as well
as some of the visuospatial and body image disturbances, described below in patients with
right posterior lesions.
• One of the most extensive investigations of somatosensory changed after brain injury was
that of Semmes et al (1960). This work summarizes a good deal of the work of this group on
the effects of penetrating missile wounds and it is difficult to do justice to it in a brief
summary. One major finding worth recording is the fact that this group found bilateral
deficits on occasion in patients with left hemisphere lesions but only contralateral deficits
after lesions of the right hemisphere. The differences were not statistically significant and to
date there has been little or no confirmation of this hemispheric asymmetry. Semmes et al
also reported bilateral difficulty in tactually guided learning in patients whose primary
sensory deficit seemed restricted to one hand.
• Corkin (1964) tested 95 patients who had undergone cortical excision for the relief of
epilepsy and compared their performance with that of control subjects on sensory
discrimination tasks and tests of tactual learning and problem solving. Her major findings
were that sensory deficits and impairments in tactile object recognition were closely related
 to lesions in the rolandic region of either side. No lasting sensory impairment was seen in
patients whose pre- and post-central gyri were spared. The deficits were usually contralateral
though some bilateral effects were produced by unilateral excisions. A wide range of
measures such as pressure sensitivity, two-point discrimination threshold and point
localization were used. More complex functions such as tactually guided learning and
problem solving showed deficits with removals in the right hemisphere regardless of location
of the lesion within the hemisphere.

Disorders of tactile perception

Judgement of stimulus orientation

• Carmon and Bechtoldt (1969) and Fontenot and Benton (1971) – tested the perception of the
direction of tactile stimulation applied to the palm of the hand. A significant proportion of
patients with right hemisphere lesions showed bilateral impairment, whereas patients with left
hemisphere lesions showed significant impairment only in the contralateral hand.
• A test of the detection of three-dimensional orientation was devised by De Renzi et al (1971)
for use in both visual and tactile modalities. Using the hand ipsilateral to the lesion, only
those with right hemisphere lesions having visual field defects were significantly impaired.
• While no sound anatomical data exists on the regions involved, there is presumptive evidence
that the right parietal lobe maybe crucially involved in this basic spatial function.

Tactile agnosia

• The term astereognosis has in the past often been used interchangeably with the term tactile
agnosia. In either case, the patients are unable to recognize objects which they feel. If the
disorder is to conform to the general concept of agnosia it should be a higher perceptual
disorder in the presence of intact primary sense modalities.
• Denny-Brown et al (1952) considered the basic disturbance to be a failure to synthesize
separate tactile sensations into the perception of form, a process they termed
amorphosynthesis. Elsewhere they considered the difficulty to be one of inability to carry out
a ‘summation of spatial impressions’ (Denny-Brown & Chambers, 1958).
• Doubt has been cast on the existence of this, as with other forms of agnosia (Teuber, 1965a,
b). Several early neuropsychological studies directed themselves in whole or in part to the
problem. Corkin (1964) found that impaired tactual recognition was seen only in patients
who also had somatosensory deficits.
• Semmes (1965) clarified the relation of sensory status to tactile agnosia in a study of left
hemisphere, right hemisphere and bilateral lesions. She found that impaired performance on
tests of tactual shape discrimination was seen in the absence as well as in the presence of
sensory defects.
• Authors of recent papers also agree that tactile object recognition difficulty can occur in the
absence of basic somaesthetic dysfunction, and that relatively normal tactile object
recognition can be seen in cases with somatosensory dysfunction. Caselli (1991b) sensibly
suggests that the term tactile agnosia, denoting a mild disorder, be reserved for cases where
difficulty with tactile object recognition occurs without significant somatosensory loss, i.e. in
sensory in the general sense of usage of the term agnosia. The term significant somatosensory
loss is critical since Maugiere and Isnard (1995), using a method employing evoked
potentials, failed to find a case of pure tactile agnosia in their large series. If cases do exist,
the lesions responsible are probably in the secondary somatosensory cortex or
parietotemporal or insular cortical regions. The term astereognosis should be reserved for a
‘lower order’ somatosensory loss, whether from cortical or spinal or peripheral nerve
damage. This form of disorder is a more severe form of deficit.

• Semmes’ earlier examination of her cases without sensory defect revealed that the
impairment was specific of her cases without sensory defect revealed that the impairment was
specific to shape, the discrimination of texture, size and roughness being unaffected. The
impairment was, however, related to spatial orientation even this was measured by a visual
task. It was suggested, therefore, that impaired shape discrimination after brain injury
depends on a general spatial factor as well as on the status of somatic sensation. Either of
these factors, seemed capable of producing impairment of tactile form discrimination but
when both were present the impairment was more severe, suggesting that the factors are
independent but additive. Semmes found that they tended to occur together in right
hemisphere lesions and she assumed that this was because of the size of the lesion, a large
lesion being needed to affect both sensation and orientation.
• The only standard psychological measure of tactual form perception is the Seguin-Goddard
formboard, which has been adapted in the much used Halstead-Reitan battery of tests. Teuber
and Weinstein (1954) showed that people with posterior lesions performed significantly
poorer on this task than those with anterior lesions, though all brain damaged subjects were
significantly inferior to controls.
 • Both Corkin (1965) and Milner (1965) used visually guided and tactually guided maze
learning in patients with variously located surgical lesions. Small parietal lesions had little
effect upon performance, while right hemisphere lesions, either frontal or temporal, produced
marked impairment. However, the complex nature of the tasks makes interpretation
difficulty.
• A study of De Renzi et al (1970) showed that tactile searching, like visual searching is poorer
for the contralateral field for both left and right hemisphere lesions but that the poorest
performances were made by the right posterior group. That the right parietal region
specifically is also involved during ipsilateral hemispace exploration was strongly suggested
by a recent PET study, using right hand exploration into right hemispace.

Unit 3: Disorders of intersensory association and spatial orientation

DISORDERS OF INTERSENSORY ASSOCIATION -

• Studies on intersensory association or cross-modal integration are of interest because such

integration or association between different forms of sense information would appear to be
basic to many higher functions. Damage to the region in which such integration is likely to
place i.e., the area of conjunction of the temporal, parietal and occipital lobes in the dominant
hemisphere has often been said to produce the most marked losses of cognitive functions.
• Butters and Brody (1968) found that patients with neurologically confirmed dominant
parietal lobe damage were particularly impaired on cross-modal matching tasks
(auditoryvisual, tactual-visual, visual-tactual), whereas frontotemporal patients were
unimpaired either on intra-modal or cross-modal tasks. Deficits on the auditory-visual
matching task were closely associated with reading difficulties, confirming the notion that
this cross-modal association is a prerequisite for reading. Many of the parietal patients who
were impaired on the visual-tactual and tactual-visual matching tasks were also impaired on
copying tests which are often used to assess constructional praxis, whereas those with mild or
no signs of parietal involvement showed little or no copying impairment. The possibility that
certain kinds of intersensory association or integrations may underlie some constructional
apraxia disorders and other voluntary motor behavior is intriguing and certainly deserving of
further study.
• Butters et al (1970a) found some impairment of cross-modal association (auditory-visual
matching) in patients with severe parietal signs but none in those with only mild parietal
signs. The care that must be exercised in interpretation of findings was highlighted by the
 subsequent finding that the impairment of right parietal patients on the auditory visual task
was associated with an auditory decoding problem rather than failure of cross-modal
association. The inferred size of the lesions and the propinquity of the auditory association
area makes it possible that the difficulty arose from disruption of function in this area than
from parietal damage.
• Kotzmann (1972) compared the performance of 10 unilateral left lesion patients with 10
unilateral right and 10 control subjects on a tactile-visual recognition task. Subjects were
asked to palpate two classes of objects and to choose the corresponding drawing from a
multiple choice visual array. One set of objects was meaningful (pipe, eggcup, bolt etc.) and
the other meaningless (moulded nonsense shapes). The brain damaged groups performed
more poorly than control subjects on both tactual recognition tasks. The
meaningful/meaningless dichotomy was not related to the laterality of the lesion in any
simple way. Left hemisphere subjects had about equal difficulty with the two tactual tasks,
while right hemisphere subjects performed more poorly on the meaningless task than on the
meaningful task.
DISORDERS OF SPATIAL ORIENTATION

The disorders within this category are those concerning the appreciation of spatial relationships
between and within objects in extra-personal space. The principal categories are:

(i) Disorders in the judgement of the location or orientation of stimuli both with respect to
each other and to the person
(ii) Impairment of memory for location
(iii) Topographical disorientation and loss of topographical memory
(iv) Route finding difficulties
(v) Constructional apraxia
(vi) Spatial alexia and acalculia

Disorders of location and orientation

• Benton (1969b) suggests that a distinction might be made between impairment of localization
of a single stimulus, which could be termed a difficulty of ‘absolute’ localization and
difficulty with the perceived spatial relations between two or more stimuli, which could be
termed a difficulty of ‘relative localization’.
 • Paterson and Zangwill (1944) referred to ‘defective appreciation of spatial relations in the
visual field with or without impairment of visual localization in the strict sense’ as
visuospatial agnosia, in other words, a dissociation between ‘absolute’ and ‘relative’
localization. They also confirmed earlier reports of a tendency to overestimate the distance of
very near objects and underestimate the distance of far objects.
• Absolute localization maybe checked by asking the patient to point to a stimulus placed in a
different part of the visual field. This test is usually failed only by grossly impaired patients.
Localization difficulty has also been inferred from other tests such as the bisection of lines.
The performance of 12 patients with parieto-occipital traumatic lesions on this bisection task
and other spatial tests was described by Bender and Teuber (1947) with the addition of a
further occipital trauma case a year later (Bender & Teuber, 1948).
• Tests of more complex spatial relations often bring out difficulties when none are apparent on
simpler tests. The multiple choice version of the Benton Visual Retention test would appear
sensitive to the subtler changes in perception of spatial relations and has been relatively
neglected in the study of parietal lesions since its introduction as a diagnostic aid.
• Da Renzi et al (1971) – devised a simple task where two rods joined together could be placed
at various orientations or angles to each other. The subject’s task was to align another pair of
rods in the same orientation under the two conditions of visual and tactile assistance. When
testing in this way they found that gross impairment was associated almost exclusively with
posterior lesions of the non-dominant hemisphere.
• A right hemisphere group was inferior to a left hemisphere group on a task by the Milan
group – who used the reproduction of the location of a number of crosses drawn at random on
paper, the measure of performance being taken from the sum of the distances by which the
subject’s copies deviated from the originals.
• Right posterior lesion patients were most impaired on a visual localization task.

Impaired memory for location

Topographical disorientation and loss of topographical memory

• Several different difficulties maybe encompassed under this heading: (i) the inability to recall
the spatial arrangement of familiar surroundings such as the disposition of rooms within the
patient’s house or the disposition of furniture within a room; and (ii) the inability to recall and
describe well-known geographical relationships with which the patient was formerly familiar.
Benton (1969b) considered the basic defect to be difficulty in calling up visual images – ‘we
 deal here with an impairment in revisualization, a failure to retrieve long-established visual
memories’. This is similar to the ‘visual irreminiscence’ of Critchley (1953).
• In 1955, Semmes and her co-workers described an objective test of spatial orientation. This
consisted of 15 diagrams of the type shown in Figure 6.6. The nine dots on each map
represented nine circles on the floor of a room, the circles being some 137cms apart. One
wall of the room was designated as North and North was marked on the maps. With the map
maintained in constant orientation to the body, the subject was required to walk around each
designated path. In this way, the orientation of the person to the room was constantly
changing. Both visual maps and ‘tactile-only’ version were provided. The performance of
parietal lobe cases was significantly inferior to that of non-parietal patients and control
subjects. There was no difference between non-parietal patients and control subjects.
Furthermore, as the disorder proved to be unrelated to perceptual modality (visual or tactile),
Semmes and her colleagues felt that it was incorrect to label the difficulty as part of
visuospatial agnosia. A second study (Weinstein et al, 1956) confirmed the poor performance
of parietal subjects on the route finding task but in this study poor performance was also
shown by frontal subjects though to a lesser degree. These frontal subjects also performed
poorly on a test of personal orientation.
With regard to the locus of the lesion, both personal and extra-personal orientation were impaired
by lesions of the posterior part of the left hemisphere. Anterior lesions (particularly those of the
left hemisphere) tended to impair personal but not extra-personal orientation, whereas the
converse was the case for right posterior lesions.
• Two studies cited in the previous section (Butters & Barton, 1970; Butters et al, 1970) have
shown that parietal patients have difficulty with reversible mental spatial operations while
other patients do not. Butters, Soeldner and Fedio (1972) extended these findings by
including a test which cannot be performed by rotating an external object either at the
concrete or the abstract level. In the test, Money’s Standardized Road Map Test of Direction,
patients need the ability to rotate themselves in imagery or on an abstract level. With the road
map in a fixed stationary relationship they are required to describe the right or left turns
which would need to be made while following the route.
• A comparison of left frontal and right parietal patients on this task allowed a test of Teuber’s
hypothesis that the frontal and parietal regions mediate qualitatively different spatial abilities:
(i) spatial orientation to external objects mediated by the parietal regions, particularly the
right; and (ii) spatial discrimination involving the subject’s own body mediated particularly
 by the left frontal region. The study demonstrated a partial double dissociation between the
two groups on the test of personal and egocentric space (Money’s test) and a test of extra
personal space (the Stick test described above).
• It has become evident that the relation between locus of lesion and topographical loss is less
clear than with some other ‘spatial’ disorders, probably because of the complexity of the
notion. The most frequent reports have been after posterior lesions – right, left or bilateral –
though pure cases are probably more common with right parietal or right parahippocampal
lesions. Topographical disorientation is particularly associated with infarcts of the right
posterior cerebral artery producing lesions in the medial aspect of the temporo-occipital
cortex.

•
 •
With regard to the locus of the lesion, both personal and extra-personal orientation were
impaired by lesions of the posterior part of the left hemisphere. Anterior lesions (particularly
those of the left hemisphere) tended to impair personal but not extra-personal orientation,
whereas the converse was the case for right posterior lesions.
• Two studies cited in the previous section (Butters & Barton, 1970; Butters et al, 1970) have
shown that parietal patients have difficulty with reversible mental spatial operations while
other patients do not. Butters, Soeldner and Fedio (1972) extended these findings by
including a test which cannot be performed by rotating an external object either at the
concrete or the abstract level. In the test, Money’s Standardized Road Map Test of Direction,
patients need the ability to rotate themselves in imagery or on an abstract level. With the road
map in a fixed stationary relationship they are required to describe the right or left turns
which would need to be made while following the route.
• A comparison of left frontal and right parietal patients on this task allowed a test of Teuber’s
hypothesis that the frontal and parietal regions mediate qualitatively different spatial abilities:
(i) spatial orientation to external objects mediated by the parietal regions, particularly the
right; and (ii) spatial discrimination involving the subject’s own body mediated particularly
by the left frontal region. The study demonstrated a partial double dissociation between the
two groups on the test of personal and egocentric space (Money’s test) and a test of extra
personal space (the Stick test described above).
• It has become evident that the relation between locus of lesion and topographical loss is less
clear than with some other ‘spatial’ disorders, probably because of the complexity of the
notion. The most frequent reports have been after posterior lesions – right, left or bilateral –
though pure cases are probably more common with right parietal or right parahippocampal
lesions. Topographical disorientation is particularly associated with infarcts of the right
posterior cerebral artery producing lesions in the medial aspect of the temporo-occipital
cortex.

Route finding difficulties

• The neurological literature has occasionally reported cases where the principal deficit lies in
the patient’s ability to find their way about in long familiar surroundings or in locations
frequently encountered in the recent past. Such patients maybe well able to give adequate
verbal descriptions of familiar routes but are unable to execute them either by drawing or by
 •
taking them in real life situations. They become lost in the hospital environs or en route to the
hospital.
Perhaps for lack of a more appropriate term such a disorder is often termed topographical
amnesia. Brain (1941) pointed to atleast four basic disorder which might lead to route finding
difficulties: (i) perceptual disorders of the location and relative position of objects; (ii) failure
of recognition of environmental features through object agnosia; (iii) topographical amnesia;
and (iv) unilateral spatial neglect. This latter cause may result in the patient failing to take
appropriate left-hand turns with a resulting preponderance of right-hand turns.
• Topographical agnosia is the inability to recognize objects that serves as landmarks.
Topographical amnesia: Cases where amnesia for topography forms the sole or major
complaint are likewise rare. The amnesia may include long stored topographical information
as well as material more recently acquired, e.g. Paterson and Zangwill’s patient (1945)
displayed an inability to describe previously experienced topographical relationships and
could not recall whether to turn right or left when leaving the ward doorway to visit the toilet.

Unit 4: Constructional Apraxia, Spatial Alexia and Acalculia

• After first considering certain drawing difficulties of neurological patients to be a form of

optic apraxia, Kleist later introduced the term ‘constructional apraxia’.
• Moreover, Benton (1969a) pointed out that Kleist insisted that this particular disorder was to
be distinguished from others that were obviously rooted in visuoperceptual disorders and
thought that the basis of the disorder lay in a disruption between visual and kinaesthetic
purposes – quite similar to the idea of ‘disconnection syndrome’.
• Many early investigators had felt that the failure of patients on certain tasks such as drawing
and route finding reflected a visuospatial disturbance. While many patients with
constructional difficulties did have visuospatial problems, there were also those who had, for
example, severe difficulties with drawing without showing a spatial deficit.
• Strauss (1924) defined a pure case of constructional apraxia as one with adequate form
perception, perceptual discrimination and perceptual localization and absence of ideomotor
apraxia or motor disability.
• Benton (1967) defined it as ‘an impairment in combinatory or organizing activity in which
details must be clearly perceived and in which the relationships among the component parts
of the entity must be apprehended if the desired synthesis of them is to be achieved’.
 •
Constructional apraxia is often said to be the apraxia of the psychologist, since it is more
often revealed on neuropsychological examination than clinically exhibited.

Status of the concept

One of the most frequently asked question is the following: is constructional apraxia a single
entity or are there separate, distinct types of disorder under this heading? In 1957, Ettlinger
and his co-workers reported 10 cases with right posterior cerebral lesions who showed
disturbances in spatial perception and manipulation. An investigation of individual
performances suggested the possibility of different varieties of constructional difficulty.
• Constructional praxis is a broad concept which has been applied to a number of rather
different types of activities. These activities have in common the characteristic that they
require the patient to assemble, join, or articulate parts to form a single unitary structure.
However, the may differ from each other in many respects, e.g., in complexity, in the type of
movement and the degree of motor dexterity required in achieving the task, in the demands
made on the higher intellectual functions, and in whether they involve construction in two or
three spatial dimensions.
• Critchley (1953) pointed out that three dimensional construction tasks appeared to be
necessary, since some patients with parietal lesions who had no difficulty on the commonly
employed two-dimensional tests displayed gross abnormalities of construction when the test
moved into the third dimension. Such abnormalities could not be elicited by clinical
examination usually.
• In a later study, Benton (1967) addressed himself specifically to the question and since it
appears to be the only such study, it is worth referring to in a little detail. Benton employed
four apparently dissimilar tests – Benton Visual Retention Test, Stick Construction, Block
Designs, Three-Dimensional Constructional Praxis – and measured the concordance between
various combinations of tests. There seemed to be more relation between the last three tests
than there was between any of these and the drawing test (VRT). Benton concluded that there
maybe atleast two types of tests, namely, graphic tasks and assembly tasks and that empirical
study may well reveal others.
• The variety of tests which have been used to elicit constructional apraxia is great:
1. Spontaneous or free drawing: for careful and detailed description with illustration of the
common errors seen with drawing tasks the read is referred to Washington (1969).
•
2. Drawing from a model, e.g. Benton Visual Retention Test as well as simpler clinical
versions.
3. Stick-pattern constructions, e.g. Stick test
4. Block design, usually modelled on the Kohs test
5. Test of spatial analysis
6. Three-dimensional constructions
 7. Reconstructions of puzzles, e.g. Benson and Barton (1970) and the Object Assembly subtest
of the WAIS

Laterality and constructional apraxia

• Most early cases described as showing constructional apraxia had bilateral posterior lesions.
Later, cases were described where constructional apraxia was seen together with one or more
symptoms of the Gerstmann syndrome, which suggested that the left or dominant parietal
area was of importance as part of the substratum of constructional abilities. Still later,
attention began to be paid to the role of the minor hemisphere when it clearly demonstrated
that unilateral right hemisphere lesions could produce the symptom.
• The first large systematic survey of laterality of lesion and constructional apraxia was that of
Piercey et al (1960) – of 403 consecutive cases with unilateral cerebral lesions, 67 cases
showed constructional apraxia. Of these, 42 had lesions on the right and 25 on the left. Ever
since, most studies have confirmed the much higher incidence of constructional apraxia with
non-dominant hemisphere lesions.
• Many studies have reported that constructional apraxia is not only more frequent with right
hemisphere lesions but that it also tends to be more severe, i.e. patient show more grossly
defective performances. However, other well controlled studies have not shown hemispheric
differences, particularly where the severity of general psychological deficit has been taken
into account.
• The assumption of a true relationship between lesions of the non-dominant hemisphere and
constructional apraxia has been seriously challenged by the study of Arrigoni and Da Renzi
(1964). While they also found a higher incidence of difficulties in right-sided lesions on each
of three consecutive tests in an unselected sample of hospitalized patients, they felt that at
least part of the difference might lie in the possibility that the lesions of the right hemisphere
group were consistently larger.

Qualitative differences and laterality

Relation between visuoperceptive and visuoconstructive deficits

• Numerous studies including that of Arrigoni and De Renzi (1964) show characteristic
differences in quality of performance on constructional tasks between left and right
hemisphere cases.
 •

• The study of Warrington and her colleagues was directed to an examination of specially
designed drawing tests which were rated by independent judges. Though there was no
significant difference in the degree of disability between the two groups, they showed a
dissociation of predominant types of error.
• The type of errors made by patients with right hemispheric lesions suggests that these
patients have difficulty in incorporating spatial information into their drawing performance,
leading to disproportion and faulty articulation of parts of the drawing, while the patients
with left hemispheric lesions produced simplified drawings, which were helped by the
presentation of a model for copying, while the visuospatial disturbances of the right
hemisphere cases were not helped to any degree by a model.
• Both Warrington (1969) and Benson and Barton (1970) have put forward hypotheses about
the relative contributions of the left and right hemispheres to visuoconstructive tasks: right
hemisphere lesions produce disorders of visuo-spatial perception while left sided lesions
disturb motor function apraxia – this suggests that right hemisphere supplies a perceptual and
the left hemisphere, an executive component to the task. The argument that the right
hemisphere contributes a perceptual element is supported indirectly by studies showing that
purely perceptual tasks (without a constructional component) are more poorly performed by
patients with right hemisphere lesions.
• On the other hand, there are studies claiming evidence that the correlation between perceptual
and constructional difficulties is higher in right than left hemisphere lesion patients. Results
of constructional tests in a small number of patients who had undergone cerebral
commissurotomy have been cited as additional evidence of the greater role of the
nondominant hemisphere in constructional performance. Though commisurotomy subjects
may perform better when using the left hand (right hemisphere) than the right hand, the fact
that performance was below par with either hand suggests bilateral but unequal
representation of constructional abilities.

Constructional apraxia and locus of lesion

• In a study of 105 cases with tumors of the temporal lobe, Petrovici (1972) found a complete
absence of constructional apraxia in 77 cases which were completely restricted to the
temporal lobe, or in 4 frontotemporal cases. On the other hand, the remaining 24 cases with
added involvement of the parietal lobe or the parietal and occipital lobes had 11 cases of
constructional apraxia.
 Bilateral parietal or diffuse cerebral lesions lead to very marked disturbances both of
constructional praxis and spatial orientation, though they are often masked by accompanying
organic dementia.

Associated difficulties

• The most widely quoted study of disabilities associated with constructional difficulties is that
of McFie and Zangwill (1960). They compared the visual-constructive impairment of 8 left
hemisphere cases (all in the posterior parietal region with that of right-sided cases.
• It is clear that there is a strong association between the first five symptoms and right-sided
lesions, while the last two symptoms are seen almost exclusively with left sided lesion.
McFie and Zangwill felt that their left-sided cases corresponded more closely to the classical
description of constructional apraxia, whereas the difficulties encountered by the right
hemisphere patients were more closely associated with visuospatial agnosia.
• As well as differences in associated symptoms, there also appeared to be qualitative
differences in the constructional disabilities of left and right cases. Failure of a high
proportion of left-sided cases on Weigl’s sorting test appeared to be part of a general
intellectual impairment which was not seen with right-sided cases. Gainotti and colleagues
(1972a) confirmed this relationship. In right-sided cases there was relation between
constructional apraxia and mental impairment, while the left-sided cases there was a
significant relationship between constructional apraxia, mental impairment and ideomotor
apraxia.

Frontal apraxia

Constructional difficulties with anterior lesions have been reported sporadically since the early
finding of Pollack (1938) that frontal lesions may occasionally disturb constructional praxis. Luria
and Tsvetkova (1964) summarized the difference between anterior and posterior lesions –

In lesions of the parieto-occipital part of the brain the general factor underlying constructive
disturbances is a loss of spatial organization of the elements…in lesions of the frontal lobes, the
general factor underlying constructive disturbances is a loss of programming and regulating of
sequential behavior…instability of the primary intention or program and to the inability to compare
the results with the preliminary intention.
 •

Constructional apraxia as a disconnection syndrome

In a largely neglected paper, Nielsen (1975) drew attention to the marked similarity in the drawing
errors of patients with right and left hemisphere lesions and the errors made by the left and right
hands of commissurotomy patients. This led to the postulation that the observed constructional
difficulties in patients with unilateral lesions might be explained at least partially by a disconnection
effect.

Independently, Le Doux and his colleagues (1978) put forward the same argument based on
observation of a patient with complete commissurotomy. This patient, while showing equal
perceptual ability of either hemisphere for block design matching showed a clear inferiority of the
right hand (left hemisphere) in executing the block design problems compared with the left hand
(right hemisphere).

According to such a model, left-sided lesions may produce constructional difficulties in the right
hand (but not the left) since they disconnect the vital right posterior region from the left motor cortex
controlling the right hand. It could be postulated that the right posterior cortex contains the basic
module for spatial integration, a plausible position in view of accumulated neuropsychological
evidence.

It would thus be possible to have three-situations:

(i) A lesion at A would produce only right handed constructional problems

(ii) A lesion at B would result in constructional difficulty for either hand
(iii) A lesion at C would produce left-handed difficulties only

Spatial alexia

• This form of alexia maybe clearly distinguishable at times from alexia of a symbolic nature.
The patient can recognize letters and words but maybe unable to read. Atleast part of this
difficulty is attributable to difficulty with the continuous scanning movements necessary for
reading.
• The disorganization in the directional control of eye movements varies a good deal from
patient to patient, but in severely disabled patients the fixations appear to be made at random,
so that fixation may jump from one part of a line to another and form part of one line to
another line some distance away.
 • Obviously a patient with this difficulty cannot make sense of printed material. Less-severely
affected patients may skip only occasional words so that they are able to fill in the sense of
what they are reading.
Some patient’s reading difficulties appear to depend largely upon unilateral spatial neglect.
For this reason, spatial alexia is seen rather more frequently with right hemisphere lesions.

Spatial acalculia

Hecaen and his colleagues divided calculation difficulties into three types, where each is the product
of a different mechanism. These are:

(i) Acalculia based on an alexia of figures and numerals (ii) Inability to do arithmetical sums
(anarithmia) (iii) Acalculia of the spatial type

Hecaen and Angelergues (1961) found a predominance of left hemisphere lesions with the first two
types and a predominance of right hemisphere lesions with the third type.

• Grafman et al (1982) studied 76 subjects with unilateral damage resulting from focal
haemorrhage, having first established the subject’s competence to read and write numbers.
They were then given a written calculation test, together with neuropsychological tests
covering perception, construction and intelligence.
• Both right and left hemisphere subjects were poorer than controls, but those with left
posterior lesions were particularly impaired even after the calculation test score had been
corrected for the results on the other tests. This would seem to confirm the presence of the
type 2 acalculia and relate it to the left posterior regions. Three similar cases of Takayama et
al (1994) implicate the region of the left intraparietal sulcus.

Unit 5: Unilateral spatial neglect (USN)

As early as 1876, Jackson described a patient who neglected the left side of the page when reading.
As with so many later reports, the lesion was in the right posterior region. A detailed description by
Brain (1941) reported inattention for the left half of space in three patients with large right-sided
parietal lesions but the condition attracted much attention.

It has been variously defined, but the essential features are expressed in the following definition
of Gainotti and his colleagues (1972b) – the syndrome consists of a tendency to neglect one half
extrapersonal space in such tasks as drawing and reading which requires a good and symmetrical
exploration of space. Testing for neglect
 •

• Simple clinical tasks include asking the patient to copy a simple symmetrical drawing such as
a daisy. The patient will often be satisfied with drawing half the figure, usually the right.
Asked to bisect a line drawn on a paper placed directly in front, the patient will place the
mark towards the normal side. This test was first devised by Axenfeld in 1894.
• However, care must be taken in reporting results of such tests to provide specific procedural
details, since the degree of neglect shown on such a task as line bisection can be affected by
factors like spatial motor cues, such as the starting position of the hand. Body or trunk
orientation has likewise been shown to affect certain neglect tasks.

Laterality and USN

• The first clear statement on a relation between laterality and neglect also came from Brain
(1945) – who considered that the deficit was very largely restricted to right hemisphere on
non-dominant lesions.
• Brain was aware that the apparently low incidence of report of USN in left hemisphere or
dominant lesions might be caused by the masking effects of other symptoms such as severe
aphasia.
• There are a number of factors which make comparison studies exceedingly difficult if not
impossible. Chief among these are the characteristics of the groups under study: e.g. (i) the
presence and degree of sensory deficits; (ii) the presence and degree of aphasia; (iii) the
presence of intellectual deterioration; and (iv) the relative size of the lesion.
• A specific test of the laterality hypothesis by Gainotti (1968) using a battery of simple tests
showed that unilateral neglect is both significantly more frequent and more severe in patients
with lesions of the right hemisphere than of the left. In order to account for qualitative
differences – Gainotti and co-workers (1972a) compared patients with unilateral lesions on
various tasks of copying drawings. They described for the first time what appears to be a
unique feature in the performance of some right hemisphere cases, namely, ‘the tendency to
neglect one half of a figure (on the side opposite to the hemispheric locus of lesion), while
reproducing designs that are placed even more laterally on the neglected side’.
• The majority of earlier references to neglect phenomena were concerned with visuospatial
neglect. It can also occur in other modalities. The finding of unilateral neglect in the tactile
modality by De Renzi and colleagues (1970) suggested to those authors ‘that hemi-attention
does not depend so much on perceptual and motor factors as on a mutilated representation of
space’.
 Heilam and Valenstein (1972a) found 17 cases of auditory unilateral neglect in a 10 month
survey. Of 10 cases with positive brain scans, 9 lesions were in the right inferior parietal
lobule and one in the left frontal region. Though the defect became most apparent in test
situations employing simultaneous stimulation, it was not restricted to these, being apparent
as an abnormal responsiveness when the patients were addressed from the neglected side.
• There was no loss of auditory acuity in these patients. There may also be an interaction
between the nature of the task employed and the laterality of the lesions.
• Leicester et al (1969) found that not only did neglect appear with lesions of either hemisphere
but also that, with particular tasks, neglect occurred predominantly or exclusively with
lesions of one hemisphere, e.g. a language related task of matching letters to an auditory
sample showed neglect only on the right with dominant left hemisphere lesions.

Locus of lesions causing neglect

Although most studies of USN implicates the posterior parts of the brain, with particular emphasis
on the non-dominant parietal lobe, neglect phenomena maybe seen with lesions in other locations,
both cortical and subcortical.

Parietal lobe

• Two studies showing evidence for role of parietal lobe came from neurosurgical reports and
localization studies using computerized tomography –
• In patients who had undergone surgery for epilepsy, Hecaen et al (1956) found the critical
area to be the supramarginal and angular gyri (the inferior parietal lobule) and part of the
adjacent superior temporal gyrus. This was confirmed by the study of Heilman and Watson
(1977) – which showed radionucleotide uptake to be maximal in this region in 14 out of 19
cases with neglect.
• Similarly the CT scan study of Bisiach et al (1979) implicated the multimodal cortex at the
junction of the parietal, temporal and occipital lobes with heavy emphasis on the inferior
parietal lobule.

Frontal lobe

• The first description of frontal lobe neglect was by Heilman and Valenstein (1972b). All 6
patients were right-handed and had right-sided lesions, 3 dorso-lateral and 3 medial. More
recently, Damasio and his associates (1980) described 5 cases of neglect with lesions in the
 •

frontal lobe or basal ganglia. In this study, 4 of the 5 cases were in the left hemisphere. That
frontal neglect is not uncommon was confirmed by Ogden (1985).
• While contralateral neglect was more severe with right-sided posterior lesions, some neglect
was also seen with about half her patients with left-sided lesions, being more common with a
frontal locus.

Subcortical lesions

• A number of reports of neglect and extinction phenomena have been reported with
subcortical lesions in the thalamus or basal ganglia. The higher incidence of impairment of
patients with right hemisphere lesions on tasks of complex visual perception, together with
the higher incidence of neglect with right-sided lesions, has suggested the hypothesis that the
two are causally related.
• Two experiments by Gainotti and Tiacci (1971) – add weight to the hypothesis that some
perceptual difficulties are caused, atleast in part, by unilateral spatial neglect. On an
overlapping figures test, right hemisphere patients made more total errors as well as more
errors and omissions for figures lying to one side (left) of the midline than the left
hemisphere group, while there was no significant difference between the two groups for
errors on figures lying to the right of the midline.
• Furthermore, the right hemisphere group with neglect tended to overvalue drawings on the
right half of the midline when asked to compare the size of two figures, one to the left and
one to the right. The authors suggest that the overvaluation may result from a tendency to
gaze to the right (non-neglected) side invoking Piaget’s theory of the ‘fixation effect’ (the
space of visual perception is not homogeneous, but the elements on which the gaze is mostly
fixed are systematically overvalued).
• A much higher incidence of visual field defects has been found in right hemisphere groups,
with a higher incidence of visual spatial neglect. There is also a generally poorer performance
on visual perceptual tasks by patients with right hemisphere lesions and neglect than by those
with right hemisphere lesions without neglect.
• The role of visual field defects in the disorders of visual perception which are so often
associated with unilateral neglect has been the subject of frequent study. The Milan group has
produced many studies showing that the perceptual deficits of non-dominant lesions are
almost always associated with visual field defects.
 The relation between field defects and neglect is far from simple. Vallar et al (1991) have
shown by the use of evoked brain potentials that early sensory processing of material from
the ‘neglected’ side does take place in some subjects at least, though this information is not
accessible to consciousness.

Nature of the defect

Four important classes of conceptions or theories with much overlap helps to explain most of the
opinions.

1. Sensory synthesis:

Denny-Brown and his colleagues (1952) considered that a disruption of sensory transmission and
synthesis in one hemisphere was the causative process. Battersby et al (1956) felt that mental
deterioration needed to be superadded.

2. Attentional deficit:
• Critchley (1949) in a discussion of tactile inattention argued strongly for an attentional deficit
as the basis of the disorder. Heilman and associates – pointed out that all the anatomical
structures described as producing neglect have been shown to be involved in arousal or
activation and attention and supported their argument with experimental studies in animals.
• More recently, this group trained subjects who were being investigated for the possibility of
epilepsy surgery on a non-verbal tactile attention task and retested them after either left or
right hemisphere sodium amytal ablation. Subjects with right hemisphere ablation showed
more extinction responses and greater inattention than those with left hemisphere ablation.
Again, these authors argue that this result arises from a greater dominance by the right
hemisphere for attentional mechanisms for scanning the environment.
3. Internal representation of space
• Neither of the above explanations accounts for the difficulties which some neglect patients
appear to have with the internal representation of space. This behavior is suggestive of a
deficit not restricted to perception but one also involving a mutilated mental representation of
space.
• This latter expression had also been used earlier by De Renzi, Faglioni and Scott (1970) who
were impressed by the fact that neglect was not restricted to the visual modality only, but
•

affected the tactile modality as well. Some of their subjects confined their tactile exploration
to the right (ipsilateral) side as though the left side of space did not exist for them.
 •

Perceptual scanning as a contributing factor for an inability to deal with the left side of
spatial representation came from an experimental study using laboratory apparatus was
conducted in a study by Bisiach et al (1981). The study employed only right-lesioned
patients, and a replication with the same test using also left-lesioned patients and a
replication with the same test using also left-lesioned subjects demonstrated the same
difficulty in both groups. The fact that left sided lesions showing neglect tend to be anterior
conforms with the evidence of the role of this region (as well as the right posterior cortex) in
spatial representation.

Neuronal network asymmetry:

• Heilman and his colleagues have cited in several papers a suggestion about a hypothesis that
the attentional neurons of the right parietal region may have bilateral receptive fields whereas
those of the corresponding left hemisphere have only contralateral fields.
• Such an explanation might account for the asymmetry of contralateral neglect supported by
the literature, but also suggests that ipsilateral neglect might also be found with right-sided
lesions.

Unilateral neglect and recovery of function:

• The more striking features of hemineglect after cerebrovascular lesions tend to ameliorate in
the first few weeks, but may remain relatively stable thereafter. Follow up studies suggest
that persistence of neglect maybe a key factor in the lesser degree of improvement in
independence and social adjustment shown by left hemiplegics compared with right.
• Despite this poor prognosis, there are those who feel that neglect maybe amenable to
rehabilitation and rehabilitative techniques have shown some promise of transferring
improved scanning from example, from training to everyday situations.

Motor neglect:

• Many patients with unilateral (sensory) neglect also may fail to use their contralateral arm,
although they are able to do so when pressed and sow no obvious motor defect when they do.
• However, Laplane and Degos (1983) have described 20 cases of ‘pure’ motor neglect, i.e.
without sensory neglect. The motor neglect was always contralateral. 15 cases were frontally
located, 4 in the parietal region and 1 was thalamic. In another series, all directional motor
 •

neglect patients had frontal lesions while in those with perceptual neglect, the frontal lobes
were spared.
Umilta (1995) points out in his review that not only may there be a dissociation between
perceptual and motor neglect, but also that sensory neglect may show a dissociation between
modalities such as visual and tactile. Even motor neglect itself maybe further subdivided –
Heilman et al (1993) prefer the term ‘action-intentional disorders’, and outline a number of
subtypes which include motor impersistence, motor extinction, akinesia and hypokinesia
while Simon et al (1995) recommend a division based on the hemispace in which the
required task is to be performed.

Unit 6: Disorders of body schema

• Disorders of the body schema are usually attributed to impairment of parietal lobe function.
Some observations would suggest that these disorders are more prominent with right parietal
lesions than with left. Absence of the disorders of communication seen with left hemisphere
lesions at least make the body schema disorders caused by right hemisphere damage appear
more apparent and striking.
• There are often associated motor and sensory deficits from spread of the lesions to
somaesthetic, visual and motor pathways. The first systematic studies of disorders of ‘the
body image’ were published by Lhermitte.

Only a sample of the most frequently seen syndromes will be outlined and theses are:

1. Anosognosia:
• This term means a failure to perceive illness. The normal clinical usage implies a failure to
perceive a defect or the denial of a defect. This association between denial or imperceptions
of hemiplegia is a very common finding and in the majority of cases, the paralysis is on the
left side. While the lesion is usually centred in the right parietal lobe, it is seldom restricted to
this site, the lesions with anosognosia being invariably large.
• Patients with this disorder may rationalize about their failure to use the paralyzed limbs and
sometimes even have the delusion that the limbs do not belong to them, i.e. they are seen as
being outside the patient’s own body image. Anosognosia is seen in the acute stages of a
disorder, being quite uncommon in chronic hemiplegics.
• The term anosognosia has also been extended by some writers to the denial or imperceptions
of other deficits. It is interesting to note that these phenomena may sometimes be dissociated
 with unawareness or denial of one deficit but not another. Recent studies of Alzheimer’s
patients, where lack of recognition of cognitive defects is very common, strongly suggest that
dysfunction of the right frontal lobe maybe the key determining factor. The non-recognition
of a defect ranges from minimization through apparent lack of concern to frank denial.

• Anosognosia for hemiplegia (Babinski’s syndrome) is almost always associated with acute,
massive vascular lesions affecting the retrorolandic area with accompanying hemiplegia,
hemianopia and hemianesthesia. The middle cerebral artery is most often implicated as the
seat of the trouble. Related, but somewhat less striking is the symptom of relative inattention
to one side of the body. Once again this deficit is mostly for the left half of the body. It
should be stressed that in these cases there is no weakness or paralysis though patients may
fail to move their limbs spontaneously such as not swinging the affected arm when walking.
They may neglect the left half of the body when bathing, dressing or combing their hair.
• Hecaen et al (1956) found that patients with surgical lesions in the right posterior
parietotemporal area had marked difficulties with complex sensorimotor activities including
dressing.
• One patient could dress store dummies at this work by had difficulty in dressing himself,
which may suggest that one of the fundamental deficits in dressing apraxia is unawareness of
the position of the limbs. This may be part of the general unawareness of body parts. The
socalled ‘dressing apraxia’ would appear to have a number of determinants, only one of
which is bodily attention.
2. Lack of awareness of body parts

This disturbance of the body image declares itself in the patient’s inability to name and localize
parts of their own bodies. Methods of examination include common clinical tests such as the
following:

i. Asking the patient to identify parts of the body named by the examiner or to move
the named parts.
ii. Asking the patient to identify body parts on a diagram or on the examiner. iii.
Asking the patient to move parts shortly after the examiner has touched them
iv. Asking the patient to touch one part of the body with another. This task is often known as
•

Head’s ‘hand-eye-ear’ test and is often a part of many routine screening devices for
higher function disorders; also used for determining difficulties with right-left
orientation.

One of the most commonly described disorders in this category is finger agnosia, first described
by Gerstmann in 1924. It was defined in a later publication in the following way – it consists in a
primary disturbance or loss of ability to recognize, identify, differentiate, name, select, indicate
and orient as to the individual fingers of either hand, the patient’s own and those of other
persons. Some writers would prefer a term like ‘faulty finger localization’.

3. Right left disorientation

This disorientation reflects itself in confusion between left and right for all parts of the body. It is
a complex disorder or set of disorder and its significance is still not yet fully understood. Many
factors may be involved, apraxia, aphasic and agnosic. Poeck and Orgass (1966) stressed the role
of aphasia though the deficit may be seen in the absence of clinically demonstrated aphasia.

Like the other symptoms under discussion of right-left hemisphere malfunction, minor degrees of
the disorder are difficult to establish since many normal individuals continue to have lateral
confusion in adult life without any apparent signs of cerebral impairment.

Unit 7: Gerstmann syndrome, parietal lobe and STM

• In 1924, Gerstmann described finger agnosia – and in surveying 4 different cases, found that
four of the features formed a unique constellation to which others gave the title of
Gerstmann’s syndrome. To the two symptoms mentioned above, finger agnosia and right-left
disorientation must be added agraphia and acalculia. These four deficits formed for
Gerstmann a unique constellation reflecting disturbance of the person’s knowledge of his or
her hand its use. Such a constellation appeared to result from dominant hemisphere lesions
affecting the region of the angular gyrus and adjacent part of the occipital lobe.
• The first major objective study to find against the validity of the Gerstmann syndrome was
that of Benton (1961) – who studied the concordance between a number of deficits in a group
of patients with cerebral disease, arguing that the strength of concordance should be a
measure of the viability of the concept of a syndrome –
• Systematic, objective analysis of the performance of patients with cerebral disease on seven
‘parietal’ tasks (right-left orientation, finger localization, writing, calculation, constructional
praxis, reading, visual memory) indicates that many combinations of deficits, including those
known as the ‘Gerstmann syndrome’, maybe observed.
• The syndrome appears to be no different from the other combinations in respect to either the
strength of mutual interrelationships among its elements or the strength of the relationships
between its elements and performances not belonging to it. These results hold both for
patients with diverse cerebral conditions and for those with focal lesions of the dominant
 parietal lobe. The finding are interpreted as indicating that the Gerstmann syndrome is an
artefact of defective and biased observation.
• The result of Poeck and Orgass (1966) supported the notion that the syndrome does not occur
in an isolated form. They noted that the complete syndrome was rarely observed without
aphasia which they believed was the ‘common denominator of the four symptoms’. The
performances which are disturbed in the so-called Gerstmann syndrome are closely related to
language.

• However, aphasia also produces other behavioural deficits, appearing as ‘concurrent’

symptoms. It is therefore not justified to regard the four symptoms as a natural syndrome.
They are an arbitrary partial grouping of the numerous neuropsychological disturbances
resulting from lesion of the leading hemisphere.

Taken from Kolb textbook:

• In 1924, Josef Gerstmann described a patient with an unusual disorder subsequent to a left
parietal stroke: finger agnosia, an asomatognosia described in Section 14.2 (see
Gerstmann, 1957). Gerstmann’s patient was unable to name or recognize the fingers on
either hand. This symptom aroused considerable interest, and in the ensuing years, three
other symptoms were reported to accompany finger agnosia: right–left confusion,
agraphia (inability to write), and acalculia. These four symptoms collectively became
known as the Gerstmann syndrome.
• Gerstmann and others argued that these symptoms accompany a circumscribed lesion in the
left parietal lobe, roughly corresponding to the angular gyrus (area PG). If these four
symptoms arose as a group, the patient was said to demonstrate the Gerstmann syndrome,
and the lesions could be localized in the angular gyrus. The Gerstmann syndrome is a
doubtful diagnostic tool in routine investigations, but all of the symptoms can be associated
with left parietal lesions.

Various other symptoms of left parietal lesions are illustrated in the following case history. On
August 24, 1975, S.S., an 11-year-old boy, suddenly had a seizure characterized by twitching on
the right side of the body, particularly the arm and face. He was given anticonvulsant medication
and remained free of symptoms until September 16, when he began to write upside down and
backward. S.S. was immediately referred to a neurologist, who diagnosed a left parietal
malignant astrocytoma.
Careful neuropsychological assessment revealed a number of symptoms characteristic of left
parietal lesions:

• Disturbed language function. S.S. was unable to write even his name (agraphia), had serious
difficulties reading (dyslexia), and spoke slowly and deliberately, making many errors in
grammar (dysphasia).

• Apraxia. S.S. was unable to combine blocks to form designs and had difficulty learning a
sequence of novel limb movements (see the next subsection). • Dyscalculia. He could not
perform mental arithmetic and was unable to solve even simple additions and subtractions.

• Recall. He had an especially low digit span, being able to master the immediate recall of only
three digits, whether presented orally or visually. • Right–left discrimination. He was totally
unable to distinguish left from right, responding at random on all tests of this ability.

• Right hemianopia. Probably because his tumor had damaged the geniculostriate connections,
as
S.S.’s tumor progressed, movement of the right side of his body became disturbed as the tumor
placed pressure on the frontal lobe.

• Roeltgen and colleagues commented ‘perhaps the pure Gerstmann syndrome is a rare
disorder because the lesions are rarely restricted to the area critical to this syndrome’. A
CT scan in Sukumar and Ferguson’s case showed the lesion confined to the angular
gyrus.
• In a review of the syndrome, Benton (1992) agreed that imaging has now confirmed the
presence of pure cases, albeit rare. Cortical stimulation appears to have further clarified
the situation, with pure deficits being elicited in discrete cortical areas between angular
and supramarginal gyri.

The parietal lobe and short-term memory (STM):

• Warrington and colleagues (1971; 1972) carried out work on the localization of
anatomical systems concerned with short-term memory dysfunction. One of the earliest
reported cases of a patient known as K.F received an injury to the left parietal region and
showed a marked impairment of the ability to repeat auditory verbal stimuli which
contrasted with much less difficulty with comparable visual verbal stimuli.
 • The case suggested the possibility of modality specific short term memory defects; in a
later study by Warrington and her colleagues (1971), patients with lesions in the left
parietal regions, this time employing psychological tests differentially loaded with
shortterm and long-term memory components’.

Results showed that:

(1) Long-term memory functions in audition were relatively infact while auditory STM was
impaired
(2) The disability was specific to the auditory modality, visual STM (as measured by relatively
normal decay function) being little affected.
• Additional differentiation of the STM defect came with a study of KF and one other
patient (Shallice and Warrington, 1974). Within the auditory modality two tests were
employed, one verbal (letters) and one non-verbal (meaningful sounds). The two patients
exhibited a dissociation between these two tasks showing impairment on the verbal task
but not on the non-verbal.
• Against these studies supporting modal specificity is the study of Butters et al (1970b).
These authors compared the performance of frontal and parietal cases, both left and right,
on a variety of short-term visual and auditory tasks again employing the distractor
technique. The right hemisphere group showed more severe impairement on visual STM
tasks compared with auditory while the two left-sided groups (frontal and parietal)
showed separate characteristics. Left frontal patients had predominantly registration but
not memory deficits. Left parietal patients had memory deficits.
• However, neither of the left groups showed modal specificity; visual and auditory
material both being affected.

• Butters et al (1970b) consider that their results can be interpreted ‘as supporting the
notion that the right hemisphere, especially the parietal region is involved in the
processing of visual information both verbal and patterned while the left hemisphere is
concerned with verbal material irrespective of sensory modality’.
• Since a disproportionate impairment in the repetition of verbal stimuli is the most
prominent symptom of ‘conduction aphasia’. Warrington considered that the results in
her three patients with left parietal lesions add weight to the anatomical basis of this
syndrome, which has been variously centred in the inferior parietal or temporo-parietal
region.
 MODULE 6: FUNCTIONAL ASPECT OF OCCIPITAL LOBE

Unit 1: Anatomy of occipital lobe

Occipital lobe forms the most posterior portions of the cerebral hemisphere and on the inner or
medial aspects there is a natural line of demarcation, called the parieto-occipital fissure. On the
lateral or convex surfaces there are no such gross landmarks and the occipital lobe merges into the
parietal lobe above and the temporal lobe below.

Following the discovery of the fact that the cerebral cortex varies in the cellular composition of its
layers from place to place in the hemisphere, and hence numerous attempts were made to map out
regions of the cortex having similar distinctive structure, known as Brodmann’s areas or
cytoarchitecture.

Brodmann’s method divided each occipital lobe into three areas having different cellular
composition (areas 17, 18 and 19). Area 17 borders on the calcarine fissure which is largely on the
medial aspect of the hemisphere and also covers the posterior pole of the hemisphere. This area is
known as the striate area because of the striped appearance when it is sectioned. It is in this area that
the neural fibres relaying information from the visual receptors in the retina reach their termination.
Area 17 is the primary visual cortex. It is surrounded by area 18, the parastriate region, which in
return is surrounded by area 19, the peristriate region, which borders on the parietal and temporal
lobes.

Area 18 is a secondary sensory area believed to be concerned with the elaboration and synthesis of
visual information. This area has numerous interhemispheric or commissural fibre connections with
the corresponding area in the other hemisphere.

Area 19 possesses abundant connections with other regions of the hemispheres so that it appears to
be chiefly involved in the integration of visual information with the information gathered by the
auditory and other sense systems and it unites visual information with the brain systems subserving
speech and other executive functions.
Visual pathways

• Knowledge of the basic anatomy of the visual pathways allows an understanding of the
diagnostic significance of certain common defects of vision brought about by lesions along
the pathways from the eye to the visual cortex. The lens of each eye focuses the stimulation
from the outer part of each eye’s visual field onto the inner half of each retina, while
stimulation arising in the inner half of the visual fields’ goes to the outer half of each retina.
The terms ‘temporal’ and ‘nasal’ have often been used to refer to this division of the
halffields of each eye.
• The terms ‘left’ (or right) halves of the visual field will be used since they are less ambiguous
in their reference than the terms ‘temporal’ and ‘nasal’. The fibres which relays information
from the retina are gathered together in the optic nerve which travels back to join its partner
in the optic chiasma. Here the fibres from the inner half of each retina cross over the midline
and go onto enter the contralateral hemisphere while the fibres from the outer halves of each
retina enter the hemisphere on the same side as the eye receiving the information. This means
that each eye projects visual information to each hemisphere. Furthermore, there is a good
deal of overlap between the visual fields of the two eyes so that most of the information (i.e.
that seen by the two eyes in common) is analysed by both hemispheres.
• From the optic chiasma, the visual pathways extend backward to the lateral geniculate bodies.
These bodies can be considered as special subdivisions of the thalamus. They relay the visual
information to the visual cortex, while the neighbouring structures, the medial geniculate
 bodies, relays auditory information to the primary projection area for audition situated in the
temporal lobes.
• The final part of the optic pathway is known as the optic radiation or geniculo-calcarine tract.
From the geniculate bodies, the pathway passes through an area called the temporal isthmus;
then its fibres fan out to cover the upper and outer portions of the lateral ventricles before
passing to the calcarine cortex.
• A lesion in the temporal isthmus forms a good example of the widespread effects that a small
lesion may have if it is strategically places. Not only does a small lesion in this location lead
to an interruption of the visual pathways and hence to a visual field defect, but it also leads to
somatosensory and motor changes since fibre pathways related to these functional systems
are in close contiguity in this region. Moreover, because of the asymmetry of function
between the two hemispheres of the brain, two clinical syndromes are recognizable according
to whether the left or right isthmus is damaged. With involvement of the left (dominant) side
severe aphasia may accompany the above symptoms while damage to the right
(nondominant) side may produce anosognosia or delusions of the body schema as well as the
visual, motor and sensory disturbances.

Unit 2: Cerebral blindness, hysterical blindness,

Cerebral blindness

• One of the most common causes of hemainopic defects is cerebral ischemia produced by
narrowing or occlusion of posterior cerebral artery.
• The loss is vision for the contralateral half-field is a prominent symptom of a failure of the
vertebrobasilar artery system to provide an adequate supply to the posterior regions of one
cerebrum. The blindness has often been termed as cortical blindness but the term cerebral is to be
preferred since the underlying white matter is usually involved as well as the cortex.
• The blindness is often accompanied with other neurological signs or associated disorders. It is
mostly vascular in origin, the blindness may follow a period of confusion or even
unconsciousness because of ‘vascular accident’ and testing of the patient, might be difficult
because of residual confusion.
• There may be intervals of days or weeks between the onset of delirium and visual and
neurological symptoms. Amnesic syndrome of marked severity may be present probably because
of bilateral ischaemia of the medial temporal regions supplied by the posterior cerebral arteries.
•
In patients who survive the precipitating vascular episode, recovery of at least some visual
function appears to be the rule (Silverman et al 1961, Gloning et al 1968). This recovery is so
frequent that it has led some workers to doubt the validity of earlier reports of lasting cerebral
blindness. Of the 32 surviving cases of cerebral blindness in Gloning and Hoffs very large
series of occipital lobe cases all recovered some visual function though many topped occipital
lobe cases.
• Recovery differs from case to case.
• Warrington (1984) described 5 patients recovered from acute stage of cerebral blindness
showed differing patterns of dissociation between the fundamental attributes of visual acuity,
spatial location, colour, and form perception.
• Restitution of visual function appears to take place in a typical order, Firstly, the sensation of
darkness becomes punctuated with elementary visual sensations or photisms.
• Next, the visual field becomes light but no form perception is possible. This is followed by
the appreciation of primitive movement, ie appreciation that the abject is moving, or has
moved, but not the direction or speed.
• Contours gradually emerge but are vague and unstable. Colour experience is last to return.
Even at this stage the visual processes may readily fatigue so that percept appears to blur
after some time (astenovia). Some patients go on to recover their normal vision but others
remain fixed stage of partial recovery.

Blindsight

• It was destruction of optic radiation resulted in total loss of vision in related parts or all of the
visual field. Riddoch noted that some patients retained some residual visual function
especially for movement, in the ‘blind’ area and later work confirmed the capacity of some
individuals to detect and localize stimuli within the blind field.
• Blindsight refers to those subjects who state that they are unaware of the visual stimuli even
when performing discriminations at high levels of proficiency. Such residual capacity may
improve by experience or practice suggesting a possible role for rehabilitation.
• Individuals have shown the capacity in a forced choice situation to discriminate between
wavelengths i.e. to show colour discrimination. However, this process happens without
awareness. The detection of deficits varies depending on the tasks, some writers have
•
assumed that the detection processes are mediated by retinal connections with the brain stem
which have been shown to subserve perceptual as well as ocular reflexes in mammals.
The fact that blindsight is seen in both normal subjects and in brain damaged subjects raise
concern.
• Weiskrantz suggest that brain imaging will help to determine whether there are structures
specifically devoted to conscious perception by comparing performances in the same subject
on the same task where the subject does or does not show awareness.

Denial of blindness( Anton’s syndrome)

• Patient appears indifferent to fails to recognise or even denies the existence of the defect.
• Post mortem findings of Anton’s case (1899) showed bilateral softening of brain in the
parieto-occipital regions and it was understood that the disconnection of the visual system
from other parts of the brain is a reason for the cause of denial.
• Damage to primary visual cortex alone was rare. Enial may also for incomplete blindness
such as hemianopic defects.

• There is a strong association between Anton’s syndrome and the tendency to confabulate.
Even the patients recovering from cerebral blindness who has not explicitly denied the defect
may admit to visual difficulties, but does almost casually.
• Some patients may be readily coerced into describing objects they failed to identify- a
condition termed ‘confabulatory pseudo-recognition’.
• Many patients with Anton’s syndrome have severe memory disorders because of the effect
of the lesion on inferomedial temporal lobe structures.

• Occasionally Anton’s syndrome is seen with blindness caused by lesions in the anterior
visual system, usually in association with confusion and widespread cognitive dysfunction.
• While denial may be verbally explicit some patients examined by Gloning and colleagues
admitted their blindness while they were actually under examination but shortly after again
denied visual loss. Denial occurs with other disorders such as hemiplegia(Babinski’s
syndrome) and noted on occasions after prefrontal lobectomy where the patient confabulates
or rationalises the presence of operative scars.
• Nathanson, Bergman and Gordon (1952) reported 28 cases of denial of hemiplegia in a
series of 100 cases. The denial occurred with lesions in either hemisphere though more
•
frequently with right hemisphere involvement. Many considered the denial of illness closely
resembled the mechanism of rationalisation or explaining away defect found in normal
subjects.
• Low level of intellectual functioning also seems to be related to the production of denial.
One difficulty in explaining Anton’s syndrome lies in the fact that some cases where
blindness and hemiplegia co-exist there may be denial of one disability with the acceptance
of other.
• Finally there has been at least one case of an inverse Anton’s syndrome- an individual with
diagnosis of cerebral blindness with preservation of only a small portion of the visual fields
denied perception despite being able to perform above chance on variety of tasks.

Adaptation to visual field defects

• Gassel and Williams(1963) found that ‘the visual function was little impaired, impaired
transiently or defective on few occasions in most patients and the ability to compensate for
the visual field defect was remarkable’.
• Completion Phenomenon – when patients with hemianopic defect are asked to fixate on a
point and a card is introduced with half an object deipicted on till the border between the half
drawing and the blank portion of the card coincides with the division between the patient’s
visual field and visual defect, they may see the whole object.
• Gassel and Williams suggests that hemianopic field loss functions as an extensive blind spot.
Hemianopic field is an area of absence which discovered rather than sensed, its presence is
judged from some specific failure in function rather than directly perceived.
• Visual field defects can be discovered on the occasion for the first ti,ee when a patient has to
deal with visual analysis and synthesis of configurations required for successful performance
on some psychological tests eg: Block design or object assembly.
• Gassel argues that the way in which hemianopic patients adapts to defects is similar to the
operations of visual perception in normal subject where imperfections of the eye as an optical
instrument do not interfere with the perception of the external world because of rapid eye
movements and shifts in attention, both of which are carried out without the conscious
awareness of the perceiver.
•
Hysterical Blindness

• Hysterical or malingering may form a possible problem in differential diagnosis.

• True cerebral blindness shows a lack of both menace and optokinetic reflexes where
the other conditions do not.
• The menace reflex is an eyeblink response produced by rapidly approaching the
corner of the eye with a menacing object, while the optokinetic reflex consists of
jerky eye
 movements when the patient gazes at rapidly moving series of objects such as rotating
striped drum.
• Electroencephalogram was used for diagnosis
• Most normal subjects show characterstic 9-13 cycles per second alpha rhythm in the
posterior regions of the brain with the subjects relaxed with eyes closed and this alpha
activity disappears when the visual stimulation is received.
• The posterior alpha rhythm is absent in cases of cerebral blindness, being replaced by
slow waves and there is no response in the record to opening and closing of the eyes. The
presence of a normal, reactive alpha rhythm should contradict a diagnosis of cerebral
blindness.
• Ishihara pseudo-isochromatic colour plates (simple and readily available test) was proved
useful in 4 cases by Bourke and Cole(1994).

Unit 3: Visual agnosia,

• Visual agnosia refers to a failure to recognize objects through the visual sense where there is
neither a primary sensory loss nor mental deterioration. It is relatively rare clinical
occurrence. The essence of the disorder as outlined by Freud in 1891, when introducing the
term agnosia was a disruption of the relationship between things themselves and the person's
concept of these things, objects, concepts. This notion distinguished the disorder from the
group of aphasias, where there was a disruption in the relationship between the concept of the
object and the word used to signify it.
• Lisseauer had distinguished between two classes of such recognition disorders the namely,
the aperceptive and associative forms, aperceptive agnosia occurs at the stage of processing
of the stimuli into an integrated percept while the associative type, the percept is formed, but
does not evoke the memory traces, which gave the percept its meaning.
• A number of separate forms of visual agnosia have been described as occurring in a pure
form that is in isolation from other defects, the forms which have been attracted most
attention, particularly in these recent years include visual object, agnosia, simultaneous
agnosia, or Simultanagnosia, visuo spatial agnosia, agnosia for faces or prospagnosia, color
agnosia, pure world blindness or agnostic alexia.

Visual object agnosia.

• This is a failure to recognize objects when presented via the visual perception modality with
preservation of recognition, by other modalities such as touch. Gassel notes that there is often
 some difficulty in drawing objects from memory or in describing them so that defective
visualization may play a part in these patients in terms sometimes patients with visual object
agnosia are able to use objects which they have failed to recognize, but are unable to state the
function of the object when shown.
• This clearly distinguishes this condition from a similar deficit seen in Amnesic aphasia or of
the patient while unable to find the correct name for a thing as well, able to describe its use.
Most of the authors index, the lateral aspect of the dominant occipital lobe as the source of
difficulty. Kliest on 1934 pointed to the importance of area 19 on the left side while Nielsen
felt that while left sided lesions were more often the cause of visual agnosia for objects or
mind blindness as it was called earlier, could also with lesion of the right occipital lesion, if
the lesion lay in the cortex of the second and third convolutions.
• Nielsen later considered that visual agnosia resulted from interruption of fibre connections
between both striate areas and the area of the left occipital region depicted. The hypothesis is
supported by the fact that cases with lesions of the left occipital lobe and the posterior
portions of the corpus callosum and the splenium do show agnosia.
• One of the requirements for a diagnosis of visual agnosia would be the absence of primary
sensory deficits such as loss of acuity.

Simultanagnosia.

• This form of visual agnosia consists of an inability to appreciate more than one aspect of a
stimulus configuration at a time. Single aspects can be identified and pointed out from a
stimulus array when it presented again suggesting clearly that the subject can identify and
remember single features or objects, even quite complex ones.
• Wolpert on 1924 first used the term simultanagnosia for one of number of deficits in his
stroke victim. This patient, while usually able to name correctly objects or drawings was
unable to grasp the meaning of a complex thematic picture. There is obvious overlap in the
description of cases with simultanagnosia and the relatively rare, the instances of balient
syndrome.
• Balient patient showed defective visual scanning and attention. He seemed unable to carry
out voluntary search and when an object was at the center of his attention, he failed to notice
other stimulus and unless pressed was unable to look to the periphery that the so called
psychic paralysis of gaze. If he attempted to attend to the details, he lost the whole. He was
 also able to carry out visually guided hand and arm moments (optic ataxia) and had
displacement visual attention to the right and left hemineglect.
• Kinsbourne and Warrington examine several cases with difficulty in simultaneous form
perception, with the difficulty of complex figure perception. They recorded the recognition
threshold for single and paired stimuli while the threshold simple stimuli were close to those
of normal, there was an invariable a long delay before the second stimulus was perceived.
They hypothesized that this slowness in perceiving more than one stimulus at a time might
render your visual scanning of the whole less efficient thus lessening the full appreciation of
the whole.
• It is obvious that the task can of analyzing and integrating the information from the magic
material as complex indeed, involving as it does the collaboration visual, perceptual,
oculomotor, attentional and cognitive practice. Any number of these in combination may
cause it deficit. If one considers that such complex perceptuo-cognitive acts depends on
occipito-frontal connections. It is not surprising that both frontal as well as occipital lesions
may disrupt the process.

Prospagnosia.

• Agnosia for faces. While patients with this disorder are able to recognize that a face is a face
and may be able to identify the individual features. They are unable to recognize familiar
faces as belonging to a particular friend, acquaintance or family member. In some cases that
receiving difficulties recognize the patient’s own face in a mirror. The term prosopagnosia
was introduced by Bodamer 10 years later.
• Prospagnosia rarely if ever occur as an isolated deficit. It is frequently associated with other
disorders particularly achromatosphia and visual object agnosia. However, unlike patients
with visual agnosia who fail to recognize the nature of objects that is the categories to which
they belong.
• Patients with prospagnosia have difficulty with identification within a category they clearly
recognize. This may occur not only for faces, but also for other perceptual categories, such as
chairs or cats, or even farm animals. Some patients show a lack of awareness of impaired
facial recognition while accepting the presence of other deficits.

Location of lesions.
 • In most studies, there has been direct or pre assumed evidence of bilateral lesions in the
occipital lobes. Gloning et.al reviewing six cases concluded that either a bilateral lesion was
present or a unilateral lesion was accompanied by involvement of the corpus callosum. They
reported post-mortem evidence of bilateral softening of the brain in the region of lingual and
fusiform gyri in a case which had shown prosopagnosia and other mark signs of visual
agnosia, but normal visual acuity.
• False recognition-This may follow massive damage to the right hemisphere. Rapscak et.al
suggest that while the left Hemisphere may still be able to employ a feature based analytic
strategy this is prone to error by the complex perceptual analysis, such as that in facial
recognition is largely configural in nature. False recognition together with prosopagnosia
occur in right posterior cases while false recognition without prosopagnosia tends to occur at
right frontal cases, and may be related to confabulation.

Color Agnosia.

• Color agnosia seems to comprise at least two separate entities namely visual agnosia for
colors and a defective color naming. Patients with agnosia for colors have difficulty in
identifying colors in practice. They are unable to match colors or order them in series as in
Holmgren's color sorting test with skeins of wool or if they can manage the task find it a
good deal more difficult than normal subjects. In keeping with each philosophy of the term
agnosia no primary sense disabilities apparent.
• Color agnosia is most often associated with visual field defects, particularly right
homonymous hemianopia. Disorders of color recognition have been reported more
frequently, more frequently with lesions of the dominant hemisphere. The color naming
deficit or the color naming defect is believed by some to be specific defect. The patient who
performs normally on the Ishihara and Holmgren's test is unable to name the color of an
object or to recognize it when it is given to them. Though this defect may occur as a part of
more general aphasia, it does occur with no observable language difficulty. The failure to
recognize the name when given distinguishes it from the amnestic or amnesic aphasia
described earlier. Geshwind's Hypothesis that difficulty with color naming is secondary to
language defect. If they understand the instructions, most aphasic patients usually do not
show abnormalities on test of color vision.
• Meadows sought to distinguish 3 required disorders of color sense. First Achromatosphia as
described is associated with bilateral inferiorly placed posterior lessions. There is a strong
 association with prosopagnosia the second disorder disconnection color anomia results from
disconnection of color perception from left hemisphere language function. Thirdly in aphasic
color anomia as exemplified in the paper of Kinsbourne and Warrington color. Perceptionist
perceived, but a patient has what meadows terms as an aphasia related to color names. This
term was used since the patient may have difficult, not only there with naming colors and
coloring drawings appropriately but we also fail on verbal test where this characteristic color
of a class of object is sort.

Unit 4: Visual hallucination, electrical stimulation

Visual hallucinations

Visual hallucinations are described frequently in conditions affecting the occipital lobes (Allen 1930,
Pailas et al 1965, Gloning et al 1968, Anderson & Rizzo 1994). The essential characteristics of these
hallucinations is their elementary nature, more organized visual hallucinations of people, objects and
scenes usually indicate that the excitation is arising in or has spread to neighboring regions,
particularly the temporal lobes. Complex hallucinations have also been described with frontal
pathology. (Nakajima 1991).

Elementary hallucinations or photisms have been described by a number of authors. Lhermitte 1951,
Ajuriaguerra & Hecaen 1960, Gloning et al 1968). The latter authors found 55 cases of elementary
hallucinations in their series. These were almost exclusively projected to the half-field contralateral
to the lesion and consisted of points, stars, flames, flashed, wheels, circles and triangles. A few cases
of photisms with lesions outside the occipital lobes were reported and each of these seemed to be
caused by irritation of visual pathways.

Elementary hallucinations have been well documented with focal epileptic seizures originating in the
occipital lobes (Lhermite 1951, Penfield & jasper 1954, Russel & Whitty 1955, Williamson et all
1992). Again, the sensations are referred to the collateral half-field.

Charles bonnet hallucinations

The term ‘Charles Bonnet syndrome’ was coined by De Morsier (1967) to refer to complex visual
hallucinations in elderly persons without evidence of mental deterioration. There is characteristically
preservation of insight, and cognition is generally intact even on formal testing (Schultz & Meizack
1993), though there may be some expectations to this latter finding (Cole 1992). Hallucinations do
not appear in other sense modalities.
The condition is most frequently seen in the elderly, but an analysis of literature reveals that it can
appear at any stage (Schultz & Meizack 1991). The aging subjects almost always have poor vision
(E.g Shedlack et al 1994) and a large prospective review of elderly patients with poor vision found
11% to have the bonnet phenomenon (Teunisse et al 1955). Most of the 14 patients studied by
Teunisse et al (1994) showed evidence of social isolation.
Though the condition is dealt with in this chapter, there is no suggestion that the occipital lobes are
the site of origin-this is at present unknown. Also, the implication that the condition is not associated
with pathology should be regarded with caution since there are two recent reports of patients with
the Bonnet syndrome who many months later went on to develop Alzheimer’s disease.

Electrical stimulation

The findings of studies of spontaneously occurring hallucinations in posterior cerebral lesions have
been very strongly confirmed by the results of stimulation, in that complex visual experiences, most
frequently of a person or group of persons, are produced from the temporal, temporo-occipital or
parieto-occipital regions but not from the visual cortex itself. Gloning, Glongin and Hoff (1968)
reported no hemispheric difference in the incidence of complex visual hallucinations in their lesion
cases. This is in marked contrast to the stimulation findings reported by Penfiled and Perot (1963)
where visual hallucinations of prior experience were elicited overwhelmingly from the right
hemisphere.

The content of hallucinations, when complex, may be determined by prior experience. A male
patient of Gloning and his colleagues who had been a philatelist saw postage stamps in the
hemianopic parts of his visual field while one of our patients who had been a forestry officer saw
predominantly scenes with trees. As is often the case, the hallucinations were vividly colored, the
patient describing them as colors he had not seen before ‘colors such as they bring out for new cars
each year. They were so vivid that they prevented the patient getting off to sleep. Sometimes the
experimental hallucinations elicited by stimulation is very similar to that experienced by the patient
during an epileptic seizure. The following case is condensed from Penfield and Perot (1963).

A 12-year-old boy with a three-year history of seizures. The pattern of his attacks was

1. Visual sensations (coloured triangles)

2. Experiential hallucinations- visual
3. Automatism – after the visual sensation usually he would see a robber, or a man with a gun,
moving towards him. The man was someone he had seen in the movies or the comic strips
 (stimulations of several points on the right temporo-occipital cortex produced the following
responses).

The very wide experience of Penfielf and his colleages with brain stimulation (Penfiled &
Rasmussen 1950, Penfielf & Jasper 1954; Penfield & Perot 1963) shows that lights, shadows,
colours, movements and other elementary visual sensations occur on stimulation of the occipital lobe
and never complex figures or scenes. In recent times, three patients receiving stimulations in the
medial parieto-occipital region each reported visual motor phenomenon.