Cerebrovascular Diseases

HYPOXIA, ISCHEMIA, AND

INFARCTION
• The brain requires a constant supply of glucose and oxygen, which is
delivered by the circulation.
• Although the brain accounts for only 1% to 2% of body weight, it
receives 15% of the resting cardiac output and accounts for 20% of
the total body oxygen consumption.
• Cerebral blood flow, normally about 50 mL per minute for each 100
gm of tissue
Hypoxia
• hypoxia is a state in which oxygen is not available in sufficient
amounts at the tissue level to maintain adequate homeostasis
• The brain may be deprived of oxygen by any of several mechanisms:
functional hypoxia in a setting of a low partial pressure of oxygen
(pO2), impaired oxygen-carrying capacity of the blood, or inhibition of
oxygen use by tissue; or ischemia
ischemia
• Ischemia is a vascular disease involving an interruption in the arterial
blood supply to a tissue, organ
• the survival of the tissue at risk depends on : the availability of
collateral circulation, the duration of ischemia, and the magnitude
and rapidity of the reduction of flow.
• Two principal types :
• Global cerebral ischemia occurs when there is a generalized
reduction of cerebral perfusion,
• Focal cerebral ischemia follows reduction or cessation of blood flow
to a localized area
Infarction
• Infarction is tissue death or necrosis due to inadequate blood supply
to the affected area.
• It may be caused by artery blockage, rupture, mechanical
compression, or vasoconstriction
• focal ischemia , if it is sustained, to infarction of a specific region of
CNS tissue within the territory of distribution of the compromised
vesse
INTRACRANIAL HEMORRHAGE
• Hemorrhages within the brain parenchyma
• subarachnoid space
Intracerebral (Intraparenchymal)
Hemorrhage
• Spontaneous (nontraumatic) intraparenchymal hemorrhages occur
most commonly in middle to late adult life, with a peak incidence at
about age 60 years.
• Most are caused by rupture of a small intraparenchymal vessel.
• Hypertension is the most common underlying cause of primary brain
parenchymal hemorrhage
cont...
• Hypertensive intraparenchymal hemorrhage may originate in the
putamen (50% to 60% of cases), thalamus, pons, cerebellar
hemispheres (rarely), and other regions of the brain
• . When the hemorrhages occur in the basal ganglia and thalamus,
they are designated ganglionic hemorrhages
Subarachnoid Hemorrhage
• The most frequent cause of clinically significant subarachnoid
hemorrhage is rupture of a saccular (berry) aneurysm
• Saccular (berry) aneurysm (congenital aneurysm) is the most common
type of intracranial aneurysm
• The etiology of saccular aneurysms is unknown
Clinical Features
• can be separated into acute events, occurring in the hours to days after the
hemorrhage, and late sequelae associated with the healing process.
• In the early post-subarachnoid hemorrhage period, regardless of the
etiology of the hemorrhage, there is an increased risk of injury from
vasospasm involving vessels other than those originally injured.
• This vasospasm can lead to additional ischemic injury.
• This problem is of greatest significance in cases of basal subarachnoid
hemorrhage, in which vasospasm can involve major vessels of the circle of
Willis.
• Various mediators have been proposed to play a role in this reactive
process;
Vascular Malformations
• Vascular malformations of the brain are classified into four principal
groups: arteriovenous malformations, cavernous angiomas, capillary
telangiectasias, and venous angiomas
• Arteriovenous malformations involve vessels in the subarachnoid
space extending into brain parenchyma or may occur exclusively
within the brain.
• In macroscopic appearance, they resemble a tangled network of
wormlike vascular channels and have a prominent, pulsatile
arteriovenous shunt with high blood flow through the malformation
Cavernous hemangiomas
• consist of greatly distended, loosely organized vascular channels with
thin, collagenized walls and are devoid of intervening nervous tissue
(thus distinguishing them from capillary telangiectasias).
• They occur most often in the cerebellum, pons, and subcortical
regions
• Capillary telangiectasias are microscopic foci of dilated, thin-walled
vascular channels separated by relatively normal brain parenchyma
and occurring most frequently in the pons
Clinical Features
• Arteriovenous malformations are the most common type of clinically
significant vascular malformation.
• Males are affected twice as frequently as females, and the lesion is
often recognized clinically between the ages of 10 and 30 years,
• presenting as a seizure disorder, an intracerebral hemorrhage, or a
subarachnoid hemorrhage. The most common site is the territory of
the middle cerebral artery
HYPERTENSIVE CEREBROVASCULAR
DISEASE
• The most important effects of hypertension on the brain include
• massive hypertensive intracerebral hemorrhage
• lacunar infarcts and slit hemorrhages, and hypertensive
encephalopathy.
• Atherosclerosis and diabetes are frequently associated diseases
Lacunar Infarcts
• Hypertension affects the deep penetrating arteries and arterioles that
supply the basal ganglia and hemispheric white matter as well as the
brainstem. These cerebral vessels develop arteriolar sclerosis and may
become occluded
• An important clinical and pathologic outcome of CNS arterial lesions is
the development of single or multiple, small, cavitary infarcts—
lacunes, or lacunar state
• These are lake-like spaces, less than 15 mm wide, which occur in the
lenticular nucleus, thalamus, internal capsule
Slit Hemorrhages
• Hypertension also gives rise to rupture of the small-caliber
penetrating vessels and the development of small hemorrhages.
• In time, these hemorrhages resorb, leaving behind a slitlike cavity (
• surrounded by brownish discoloration; on microscopic examination,
• slit hemorrhages show focal tissue destruction, pigment-laden
macrophages, and gliosis.
Hypertensive Encephalopathy
• characterized by diffuse cerebral dysfunction, including headaches,
confusion, vomiting, and convulsions, sometimes leading to coma.
• Patients coming to postmortem examination may show an edematous
brain with or without transtentorial or tonsillar herniation.
• Petechiae and fibrinoid necrosis of arterioles in the gray and white
matter may be seen microscopically
Brain infection
Acute Pyogenic Meningitis
• In neonates, the organisms include Escherichia coli and the group B
streptococci
• Among adolescents and in young adults, Neisseria meningitidis is the
most common pathogen
• The introduction of immunization against Haemophilus influenzae
has markedly reduced the incidence of meningitis associated with this
organism in the developed world
• Patients typically show systemic signs of infection superimposed on
clinical evidence of meningeal irritation and neurologic impairment,
including headache, photophobia, irritability, clouding of
consciousness, and neck stiffness.
• A spinal tap yields cloudy or frankly purulent CSF, under increased
pressure, with as many as 90,000 neutrophils/mm3, a raised protein
level, and a markedly reduced glucose content
Acute Aseptic (Viral) Meningitis
• absence of recognizable organisms in an illness with meningeal
irritation, fever, and alterations of consciousness of relatively acute
onset.
• The disease is generally of viral
• The clinical course is less fulminant than that of pyogenic meningitis
• In aseptic meningitis, there is a lymphocytic pleocytosis, the protein
elevation is only moderate, and the sugar content is nearly always
normal
Brain Abscess
• Brain abscesses may arise by direct implantation of organisms, local
extension from adjacent foci (mastoiditis, paranasal sinusitis), or
hematogenous spread (usually from a primary site in the heart, lungs,
or distal bones or after tooth extraction
• Predisposing conditions include acute bacterial endocarditis, cyanotic
congenital heart disease
Subdural Empyema
• Bacterial or occasionally fungal infection of the skull bones or air
sinuses can spread to the subdural space and produce a subdural
empyema.
• The underlying arachnoid and subarachnoid spaces are usually
unaffected, but a large subdural empyema may produce a mass effect.
• Further, a thrombophlebitis may develop in the bridging veins that
cross the subdural space, resulting in venous occlusion and infarction
of the brain
Extradural Abscess
• Extradural abscess, commonly associated with osteomyelitis, often
arises from an adjacent focus of infection, such as sinusitis or a
surgical procedure.
• When the process occurs in the spinal epidural space, it may cause
spinal cord compression and constitute a neurosurgical emergency.
CHRONIC BACTERIAL
MENINGOENCEPHALITIS
Tuberculosis
Patients with tuberculous meningitis usually have symptoms of
headache, malaise, mental confusion, and vomiting.
There is only a moderate CSF pleocytosis made up of mononuclear
cells
the protein level is elevated, often strikingly so; and the glucose
content typically is moderately reduced or normal.
Morphology.
• gelatinous or fibrinous exudate, most often at the base of the brain,
There may be discrete, white granules scattered over the
leptomeninges.
• The most common pattern of involvement is a diffuse
meningoencephalitis.
• On microscopic examination, there are mixtures of lymphocytes,
plasma cells, and macrophages.
• granulomas, often with caseous necrosis and giant cells.
Clinical Features
• The most serious complications of chronic tuberculous meningitis are
arachnoid fibrosis, which may produce hydrocephalus, and
obliterative endarteritis, which may produce arterial occlusion and
infarction of underlying brain
• Infection by Mycobacterium tuberculosis in patients with acquired
immunodeficiency syndrome (AIDS) is often similar to that in non-
AIDS patients, but there may be less host reaction.
Neurosyphilis
• Neurosyphilis is the tertiary stage of syphilis and occurs in only about
10% of patients with untreated infection.
• The major forms are meningovascular neurosyphilis, paretic
neurosyphilis, and tabes dorsalis
Morphology
• Meningovascular neurosyphilis is a chronic meningitis involving the
base of the brain and, variably, also the cerebral convexities and the
spinal leptomeninges.
• Paretic neurosyphilis caused by invasion of the brain by Treponema
pallidum and is clinically manifested as insidious but progressive loss
of mental and physical functions with mood alterations
• terminating in severe dementia
• On microscopic examination, inflammatory lesions are associated
with parenchymal damage in the cerebral cortex
• characterized by loss of neurons with proliferations of microglia (rod
cells), gliosis,
• The spirochetes can be, at times, demonstrated in tissue sections.
• There is often an associated hydrocephalus with damage to the
ependymal lining and proliferation of subependymal glia, called
granular ependymitis
• Tabes dorsalis is the result of damage by the spirochete to the
sensory nerves in the dorsal roots, which produces impaired joint
position sense , sensory disturbances and absence of deep tendon
reflexes.
• On microscopic examination, there is loss of both axons and myelin in
the dorsal roots, with pallor and atrophy in the dorsal columns of the
spinal cord.
Neuroborreliosis (Lyme Disease)
• Lyme disease is caused by the spirochete Borrelia burgdorferi,
transmitted by various species of tick
• involvement of the nervous system is referred to as neuroborreliosis.
• Neurologic symptoms are highly variable and include aseptic
meningitis, facial nerve palsies, mild encephalopathy, and
polyneuropathies
VIRAL MENINGOENCEPHALITIS
• Viral encephalitis is a parenchymal infection of the brain almost
invariably associated with meningeal inflammation
(meningoencephalitis) and sometimes with simultaneous involvement
of the spinal cord (encephalomyelitis).
• The most characteristic histologic features of viral encephalitis are
perivascular and parenchymal mononuclear cell infiltrates , glial cell
reactions
Human Immunodeficiency Virus
• As many as 60% of patients with AIDS develop neurologic dysfunction
during the course of their illness
HIV Meningoencephalitis
• Patients affected with this remarkable neurologic disorder can
manifest clinically with dementia and mood disturbances, such as
apathy and depression.
• Motor abnormalities, ataxia, bladder and bowel incontinence, and
seizures can also be present. Radiologic imaging of the brain may be
normal or may show some diffuse cortical atrophy
AIDS-Associated Myopathy and
Peripheral Neuropathy
• Inflammatory myopathy has been the most often described skeletal
muscle disorder in patients with HIV infection. The disease is
characterized by the subacute onset of proximal weakness,
sometimes pain, and elevated levels of serum creatine kinase.
• The most commonly reported clinical syndromes of peripheral
neuropathy include acute and chronic inflammatory demyelinating
polyneuropathy
Progressive Multifocal
Leukoencephalopathy
• Progressive multifocal leukoencephalopathy (PML) is a viral encephalitis
caused by the JC polyomavirus; because the virus preferentially infects
oligodendrocytes, demyelination is its principal pathologic effect.
• The disease occurs almost invariably in immunosuppressed individuals
• No clinical disease has been associated with primary infection by the JC
virus, but about 65% of normal people have serologic evidence of
exposure to the virus by the age of 14 years.
• It is though that PML results from the reactivation of virus as a result of
immunosuppression
• The cerebrum, the brainstem, the cerebellum, and occasionally the
spinal cord can be involved.
• On microscopic examination, the typical lesion consists of a patch of
demyelination
FUNGAL MENINGOENCEPHALITIS
• fungal disease of the CNS is encountered primarily in
immunocompromised patients.
• The brain is usually involved only late in the disease, when there is
widespread hematogenous dissemination of the fungus, most often
Candida albicans, Mucor, Aspergillus fumigatus, and Cryptococcus
neoformans.
• In endemic areas, pathogens such as Histoplasma capsulatum
involved
• There are three main patterns of fungal infection in the CNS: chronic
meningitis, vasculitis, and parenchymal invasion. Vasculitis is most
frequently seen with Mucor and Aspergillus,
• Parenchymal invasion, usually in the form of granulomas or abscesses,
can occur with most of the fungi
• Cryptococcal meningitis, observed now with increasing frequency in
association with AIDS, may be fulminant and fatal