Neuro Shelf

Defect of Shh in the prosencephalon

o Leads to Holoprosencephaly Prosencephalon fails to develop into 2 lobes of
telencephalon
Neural crest cells go on to create, among other things, Schwann cells, melanocytes, and
enterochromaffin cells
Syringomyelia
o Cape-like, bilateral loss of pain and temperature sensations in upper
extremities
alpha-fetoprotein & acetylcholinesterase can be measured as surrogates of neural tube
defects

This baby had spina bifida (small sacral tuft of hair) Folic acid is given to mother to
reduce neural tube defects Folic acid deficiency leads to megaloblastic anemia
Hypersegmented neutrophils are seen
Dermatitis in B3 deficiency
Nigh blindness Vit A deficiency
Numbness and tingling B12 deficiency
Pool wound healing Vitamin C deficiency
 Albinism Problem with melanocytes (deficiency in tyrosinanse activity) melanocytes
develop from neural crest cells, which develop from the neuroectoderm
Ectoderm develops into skin, CNS
Endoderm develops into gut epithelium, pancreas, liver, lungs
Mesoderm develops into muscle, bone, connective tissue
The non-neural crest part of the neuroectoderm develops into the brain, spinal cord

Microglia is derived from the mesoderm layer

Guillain-Barr syndrome
o From autoimmune destruction of Schwann cells
o Molecular mimicry
Vestibular Schwannoma
o Affects CN VIII
o Located on the cerebellopontine angle
Oligodendrocytes are injured in multiple sclerosis
Neurons regenerate at about 1mm/day, as long as the cell body is not damaged

The major neurotransmitter in REM sleep is Acetylcholine

Leptin is a hormone secreted by adipose cells, which inhibits the lateral area and activates
the ventromedial area. Its activity in the hypothalamus leads to feelings of satiety
o Fell full
 The cerebellar hemisphere mediates motor movements on the ipsilateral side because the
pathways cross twice. They cross once ascending the superior cerebellar peduncle and the
other time through the pyramidal decussation of the corticospinal tract in the medulla

Dopamine from the substantia nigra acts on the basal ganglia to activate the direct pathway
and inhibit the indirect pathway facilitates movement

his shows a diminished substantia nigra and the person has Parkinsons disease (the one on the
left is a normal one and the one on the right in indicative of Parkinsons disease
Lewy bodies (composed of alpha-synuclein) can be seen under the microscope
This was from a patient with Huntington disease (Diminished caudate nucleus dilation of
lateral ventricles)

The Broca and Wernicke areas are areas in the dominant hemisphere (usually left)
involved in communication.
o Broca Involved in speech production
o Wernicke For comprehension of speech

Anterior cerebral artery (ACA)
 Watershed zones are those areas of the brain most susceptible to hypoperfusion. They
occur between the ACA/MCA and between MCA/PCA
MCA strokes affects face and upper limbs
ACA strokes affects the lower limbs

Ca+2 channels blocker (vasopasm) for patients with a subarachnoid hemorrhage to avoid
vasopasm (narrowing of blood vessels), which can lead to ischemic infarct
Treatment of an ischemic stroke is with tPA, but only if within 3-4.5 hr of onset
CNs III, IV, V1, V2 and VI run through the cavernous sinus
A patient that has recently recovered from meningitis is likely to have communicating
hydrocephalus because there will be arachnoid scarring post-meningitis which is
going to impair the ability to reabsorb CSF

Tabes dorsalis is caused by 3o syphilis

o Results from degeneration (demyelination) of dorsal columns and roots
o Treatment of syphilis is penicillin G
o Argyll Robertson pupils are often seen
The pupils will constrict when the patient focuses on a near object, but
will not constrict in response to light
Horner syndrome
o Ptosis
o Anhydrosis
o Miosis
The facial nerve mediates taste from the anterior 2/3 of tongue through the chorda
tympani, whereas the glossopharyngeal nerve mediates taste and sensation from the
posterior 1/3
 Treponema pallidum is the organism that causes syphilis
Only the dorsal columns are lesioned tabes dorsalis seen in syphilis

Diminished ankle-jerk reflex

S1 root is affected Ankle-jerk is affected
Weakness to dorsiflexion is due L5 lesion, not S1
 Chromosome 4
Huntington disease (chorea, inherited)
o CAG trisomy on chromosome 4
Chromosome 18 Edward syndrome
Chromosome 9
o For frataxin in Freidricks ataxia
 Donepezil (Acetylcholinesterase inhibitors)
Alzheimer (image shows neurofibrillary tangles)
Bromocriptine (dopamine agonist for treatment of Parkinson)
Levodopa/Carbidopa for treating Parkinson
Selegiline for treating Parkinson
 Increased protein in cerebrospinal fluid
She has symptoms that have come and gone Multiple sclerosis
o Lesions are restricted to white matter of CNS
o Associated with increased IgG in the CSF due to immune response from
demyelination
Gliosis in the caudate nucleus In Huntingtons disease
Hyperphosphorylated tau protein seen in Alzheimer
Intracytoplasmic eosinophilic inclusions Lewy bodies in Parkinson

When the ciliary muscle contracts, it relaxes the lens, making it more convex. This is
important in facilitating near vision. In contrast, ciliary muscle relaxation contracts the
lens, causing it to flatten. This is important for far vision
Uveitis is inflammation of the uvea that can be caused by a number of things. The goal
of treatment is to help inflammation subside. This is done with steroids
Dont use epinephrine in a patient that has acute closed-angle glaucoma. Epinephrine
leads to mydriasis (dilation of pupil) which further closes the angle and exacerbates the
problem
Papilledema is a funduscopic sign of increased intracranial pressure
 Diabetes
Patient has CN III palsy Ptosis and down and out
This patient is experiencing motor components symptoms (which is central to the
parasympathetic fibers)
o Affected by metabolic and ischemic issues
o The parasympathetic component is affected more by trauma (Berry aneurysms,
meningitis, physical trauma)

Bronchogenic carcinoma
o A tumor here causes compression of the superior cervical ganglion
This patient has Horners syndrome
 This patient developed osmotic demyelination syndrome (central pontine myelinolysis)
as a result of rapid correction of his hyponatremia

Dementia is a gradual process leading to memory loss and progressive decline of other
cognitive abilities. Delirium has an acute onset and typically waxes and wanes
throughout the day

Guillain-Barre syndrome is associated with Campylobacter jejuni

Cluster headache
o Patient suffers from multiple episodes of unilateral periorbital pain that occurs
daily for several weeks before the symptoms subside
DIT

Basics:
Oligodendroglia
o Look like fried eggs under histologic staining
o Myelinates multiple CNS axons
o Damaged in multiple sclerosis
Microglia
o Form multinucleated giant cells in the CNS when infected with HIV
o Macrophages of the CNS
Schwann cells
o Myelinates one PNS axons
o Damaged in Guillain-Barre syndrome
Astrocytes
o Cells of the blood-brain barrier

Acetylcholine
o Decreased in Alzheimer disease
o Decreased in Huntington disease
o Increased in Parkinson disease (also loss of dopaminergic neurons in Parkinson)
GABA
o Major inhibitory neurotransmitter in the CNS
o Decreased in both Huntington disease and anxiety
Blood brain barrier
o Tight junctions between nonfenestrated capillary endothelial cells
o Basement membrane
o Astrocytes foot processes
o Lipophilic drugs commonly pass the barrier

Hypothalamus:

Suprachiasmatic nucleus
o Considered the master clock for most of our circadian rhythms
o Receives input from the retina
Anterior hypothalamus
o Regulates pArasympathetic nervous system
o Cooling (A/C) Sweating and vasodilation
o Destruction results in hyperthermia
Posterior hypothalamus
o Regulates sympathetic nervous system
o Heating (fired up sympathetic)
o Shivering and decreased cutaneous blood flow in the cold
o Destruction results in inability to stay warm
 Supraoptic nucleus
o Produces ADH to regulate water balance
o Destruction results in neurogenic diabetes insipidus
Dorsomedial nucleus
o Savage behavior and obesity from stimulation
Ventromedial nucleus
o Satiety
o Destruction leads to savage behavior and obesity
o Leptin stimulates this
Lateral nucleus
o Hunger
o Stimulation eating
o Destruction Starvation
o Leptin inhibits this
Preoptic nucleus
o Regulates release of gonadotropic hormones (LH and FSH)
Arcuate nucleus
o Releases hormones affecting the Anterior pituitary

Sleep

Stage N1theta waves

Stage N2sleep spindles and K complexes
Stage N3Delta waves
o Sleepwalking, night terrors Treat with Benzodiazepines
o Bedwetting treat with desmopressin/imipramine
REMBeta waves (same as awake state)

Narcolepsy
o Abrupt transition from awake REM
o Daytime sleepiness, cataplexy, sleep paralysis
o Treat with modafinil

Cranial Nerves

Gag reflex
o CN IX and CN X
CNs with nuclei in medulla
o CN IX
o CN X
o CN XI
o CN XII
 CNs with nuclei in pons
o CN V
o CN VI
o CN VII
o CN VIII
CNs with nuclei in midbrain
o CN III
o CN IV
Bells palsy (CN VII palsy)
o Unilateral facial drooping involving the forehead
CNs traversing through cavernous sinus:
o CNs III, IV, VI, V1
o Cavernous sinus syndrome
Ophthalmoplegia
CN III eye looks down and out
CN VI unable to look laterally
Decreased corneal sensation
Horner syndrome
Horner syndrome
o Ptosis
o Anhidrosis
o Miosis
o Associated with lesion of spinal cord above T1
Pancoast tumor
Brown-Sequard syndrome
Late-stage syringomyelia
Marcus Gunn pupil
o Due to optic nerve damage (multiple sclerosis)/retinal detachment leading to
inability of both eyes to constrict when light is shone into the affected eye

Brain Embryology

Chiari I malformation associated with:

o Syringomyelia
Chiari II malformation associated with:
o Meningomyeoloce
o Hydrocephalus
Syringomyelia
o Bilateral loss of pain and temperature sensation in upper extremities due to
compression of the anterior white commissure
o As the central canal expands, it can affect the ventral horn and cause weakness
in the hand muscles
 An Increase in alpha-fetoprotein and acetylcholinesterase in the amniotic fluid is a
helpful confirmatory test for neural tube defects
Brachial pouches
o 1st Middle and Eustachian tubes
o 2nd Epithelial lining of the palatine tonsil
o 4th Superior parathyroids
o 3rd Inferior parathyroids; Thymus

Brainstem

Anterior spinal artery

o Supply regions of the lateral corticospinal tract and medial lemniscus in the
medulla
o Occlusion leads to Medial medullary syndrome
The corticospinal tract decussates at the caudal medulla prior to the medullary
pyramids
The Dorsal column medial lemniscal pathway decussates at the medulla and then
ascends as medial lemniscus
Posterior inferior cerebellar artery
o Supply regions of the vestibular nuclei, lateral spinothalamic tract, spinal
trigeminal nucleus, nucleus ambiguus, sympathetic fibers, inferior cerebellar
peduncle
Stroke to the facial motor cortex
o Spare the upper face (can wrinkle forehead)
o Contralateral lower face droops
Bells palsy
o Unable to wrinkle both the forehead and face on affected side
Tongue innervation
o Motor Hypoglossal n. (CN XII)
o Taste
CN VII in anterior 2/3
CN IX in posterior 1/3
CN X in extreme posterior
Brainstem Lesions

Weber Syndrome
o Contralateral spastic paralysis or hemiparesis
o CN III palsy (eye looks down and out)
o Ptosis
Lateral Medullary (Wallenberg) Syndrome
o Contralateral loss of pain and temperature sensation on the body
o Ipsilateral loss of pain and temperature on the face
 o Hoarse voice, difficulty swallowing, loss of gag reflex
o Ipsilateral Horner syndrome
o Vertigo, nystagmus, nausea/vomiting
o Ipsilateral cerebellar defects

Vascular Events

Berry aneurysms
o Most common site at Anterior communicating artery
o Associated with ADPKD (Polycystic kidney disease) and Ehlers-Danlos Syndrome
(integrity of connective tissue is affected)
Subdural hematoma
o Rupture of the bridging veins
o Can develop over the course of weeks
o Cross suture lines
o Crescent-Shaped
Epidural hematoma
o Rupture of middle meningeal artery
o Not crossing suture line
o Biconvex (football shaped)
o Lucid interval
tPA
o First do CT of the head (without contrast) to determine whether the stroke is
thrombotic or hemorrhagic
o This is only administered if the stroke occurred within 3-4.5 hr and no
hemorrhage/risk of hemorrhage
Subarachnoid hematoma
o Present with Worst headache of my life
o Bloody CSF

CSF

CSF is generated by the ependymal cells of choroid plexus

o and then reabsorbed by arachnoid granulations that drain into the superior
sagittal sinus
Communicating vs Non-Communicating hydrocephalus
o Communicating
Decreased absorption of CSF by arachnoid granulations
Can be seen post-meningitis (arachnoid scarring)
o Non-communicating
Due to physical obstruction of CSF within ventricular system
 Normal pressure hydrocephalus
o Wet Urinary incontinence
o Wobbly Ataxia
o Wacky Wacky

Headaches

Migraine
o May have an aura
o Unilateral, throbbing pain (pulsating)
o Nausea/vomiting, photophobia, phonophobia
o Timeframe: 4-72 hours
Cluster
o Unilateral, non-throbbing pain
o Localized around one eye (periorbital pain)
o Lacrimation and rhinorrhea may be present
o Timeframe: 15 min- 3hr
Tension
o Bilateral frontal or occipital pain
o Tight (non-pulsating)
o No photophobia, or phonophobia
Sumatriptan
o Serotonin 5-HT1B,1D receptor agonist
o Contraindicated in patients with coronary artery disease, prinzmetal angina,
pregnancy

Brain Tumors

Most common primary brain tumors in adults:

o Glioblastoma
Pseudopalisading necrosis
Worst prognosis of any primary brain tumor
o Meningioma
Psammoma bodies
o Schwannoma
Bilateral vestibular schwannommas found in NF-2
o Hemiangioblastoma
Polycythemia
Associated with von Hippel-Lindau syndrome
Foamy cells, high vascularity
o Pituitary adenoma
Hyperprolactinemia galactorrhea, amenorrhea, anovulation
 Bitemporal hemianopsia
o Oligodendroglioma
Fried-egg appearance
Most common primary brain tumors in children:
o Astrocytoma
o Medulloblastoma
Cerebellar tumor
Can compress 4th ventricle, causing noncommunicating hydrocephalus
Homer-Wright rosettes, small blue cells
o Ependymoma
Perivascular pseudorosettes
Can cause hydrocephalus

Seizures

Partiallocalized to a specific area

o Simpleno alteration of consciousness
o Complexalteration of consciousness
Generalizedover the entire brain
o Absence blank state (day dreaming)
Treat with ethosuximide
o Myoclonic quick, repetitive jerks
o Tonic-clonic classic representation
Treat with Phenytoin, valproic acid, carbamazepine
o Tonic
o Atonic

Movement

Ia afferent pathway
o Comes from muscle spindles, which stimulates the alpha-motor neurons to get
muscle contraction
Ib afferent pathway
o Comes from golgi tendon organ, which inhibits muscle contraction
VPL thalamic nucleus
o Relays somatosensory from body (Spinothalamic and dorsal columns/medial
lemniscus)
o VPL = L= legs
VPM thalamic nucleus
o Relays trigeminothalamic and taste pathways to somatosensory cortex
o VPM = M = Makeup goes on face
VL (ventrolateral nucleus of thalamus)
o Relays from cerebellum (dentate nucleus) and basal ganglia to motor cortex
 VA (ventral anterior nucleus of thalamus)
o Relays from basal ganglia to prefrontal, premotor, and orbital cortices
Lateral Geniculate Nucleus
o Relays from retina to occipital lobe
o Lateral = Light
Medial geniculate nucleus
o Relays auditory info from brachium of inferior colliculus to primary auditory
cortex
o Medial = M= Music

UMN lesion sings

o Spastic paralysis, hyperreflexia
LMN lesion signs
o Flaccid paralysis, atrophy, fasciculations
Babinski reflex
o Toe extension upon stimulating the sole of the foot with a blunt instrument
Romberg test
o A positive Romberg test indicates that that lesion is in the dorsal columns of the
spinal cord, not the cerebellum

Basal Ganglia

Parkinson disease
o Resting tremor
o Rigidity (cogwheel)
o Akinesia
o Postural instability
o Mask-like faces
o Shuffling gait
MPTP exposure causes destruction of dopaminergic neurons in the substantia nigra
Globus pallidus internal segment
o Inhibitor of movement
o When this is disinhibited, it causes movement
Subthalamic nucleus
o Stimulates globus pallidus internal segment
o Inhibitor of movement
o Damage to this causes hemiballismus (wild flailing of arm) to the contralateral
side
Substantia nigra pars compacta
o Facilitation of movement
Huntington disease
o Decreased GABA
 o Increase dopamine
o Decreased acetylcholine
o Chorea, dementia, and atrophy of the caudate nucleus
Parkinson disease
o Depigmentation of the substantia nigra
o Associated with Lewy bodies (composed of alpha-synuclein)
Eosinophilic inclusions in the cytoplasm of neurons

Spinal Cord and Lesions

Dorsal columns
o Conveys touch, vibration, and pressure sensation
o Fasciculus gracilis & fasciculus cuneatus
Lateral corticospinal tract & Ventral
o Voluntary motor command from motor cortex to body
Corticobulbar tract
o Voluntary motor command from motor cortex to head/neck
Lateral spinothalamic tract
o Pain and temperature sensation
Vestibulospinal tract
o Important for postural adjustments and head movements
Dorsal and ventral spinocerebellar tracts
o Proprioceptive information for the cerebellum

Brown-Sequard syndrome
o Ipsilateral UMN signs below the lesion
o Ipsilateral dorsal column loss of information below the lesion
o Contralateral pain and temperature loss at 2-3 segments below the lesion
o Ipsilateral pain and temperature loss at the level of the lesion
o Ipsilateral LMN signs (flaccid paralysis) at level of lesion
ALS
o Combined UMN and LMN defects
o No sensory deficit
Multiple Sclerosis
o Scanning speech
o Intention tremor (also incontinence and internuclear ophthalmoplegia)
o Nystagmus
o Optic neuritis
Tabes dorsalis
o Caused by syphilis
o Degeneration of the dorsal columns
o Associated with Charcot joints, shooting pain, Argyll Robertson pupil
Brachial Plexus & Upper Extremity Nerves

Lower Extremity & Skeletal Muscle

Sensation

A-delta fibers
o Encode painful, sharp pain
o Fast, myelinated
C fibers
o Encode slow, longer lasting dull pain
o Slow, unmyelinated
Pacinian corpuscle
o Vibration and pressure
Meissner corpuscle
o Dynamic/changing light, discriminatory touch
Merkel receptor
o Static/unchanging light touch
Muscle spindle
o Proprioceptionmuscle length monitoring
Golgi tendon organ
o Proprioceptionmuscle tendon organ

Guillain-Barre Syndrome
o Increase in CSF protein with normal cell count
o Symmetric ascending muscle weakness (begins at the feet and moves up the
body)
o Facial paralysis (in 50% of cases)
o Preceded by infection
Eye

Open-angle glaucoma
o Peripheral vision is lost first which can progress to central vision
Drugs that can be used to treat glaucoma:
o Alpha-agonist
o Beta-blockers
o Carbonic anhydrase inhibitors
o Cholinergic agonists
o Prostaglandins
Age-related macular degeneration
o Dry
Deposition of yellowish extracellular material (fat deposits)
Prevents progression with multivitamin and antioxidant supplements
o Wet
Bleeding 20 to choroidal neovascularization
Treat with anti-VEGF

Ear

Meniere disease
o Episodic vertigo
o Tinnitus
o Sensation of pressure
Cholesteatoma
o Overgrowth of desquamated keratin debris within the middle ear space
conductive hearing loss

Delirium & Dementia

Causes of dementia that you have to screen for:

o Syphilis
o HIV
o Hypothyroidism
o Wilson disease
o Vitamin B12 deficiency
o Normal pressure hydrocephalus
Down syndrome patients are at an increased risk of developing Alzheimer because the
APP gene in the chromosome 21
Alzheimer disease
o Extracellular amyloid deposits in the grey mater
 o Neurofibrillary tangles: Intracellular deposits of hyperphosphorylated tau protein
Frontotemporal dementia (Pick disease)
o Intracellular spherical aggregates of tau protein seen on silver stain