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Cerebral vascular disease

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"cerebrovascular disease" AND "stroke"

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Cerebral Vascular Disease (Stroke and Cerebrovascular Disease)

Definition and Overview

Cerebral vascular disease (CVD) encompasses all disorders caused by pathological processes affecting blood vessels of the brain. Among all neurological diseases of adult life, stroke ranks first in frequency and importance. The hallmark is abrupt onset of a focal neurological deficit - usually a matter of seconds - that identifies the event as vascular.
  • In the United States, a stroke occurs approximately every 40 seconds
  • Prevalence increases steeply with age - approximately 15% in adults over 80
  • Stroke is the second leading cause of death worldwide and the leading cause of neurological disability in the US
  • 30-day mortality: ~10%; 1-year: ~20%; 5-year: ~40%
(Adams and Victor's Principles of Neurology, 12th Edition; Frameworks for Internal Medicine)

Classification

Broad Categories

TypeMechanismFrequency
Ischemic strokeOcclusion of a cerebral blood vessel~80%
Hemorrhagic strokeRupture with bleeding into brain or subarachnoid space~20%
Hemorrhagic stroke carries much higher acute mortality - 30-day mortality approaches 50% (5 times greater than ischemic stroke), though long-term functional outcomes among survivors are similar.
(Frameworks for Internal Medicine)

I. Ischemic Stroke

Pathophysiology

Ischemic injury results from insufficient oxygen and nutrient delivery to brain tissue. Vascular occlusion can be:
  1. Embolic - particles originating from a distant cardiovascular source; onset is abrupt and deficit peaks almost immediately
  2. Thrombotic - clot forms within a vessel near the infarct; evolves more slowly over minutes to hours, often in a saltatory (stepwise) fashion
  3. Lacunar - occlusion of small penetrating arteries deep in the brain parenchyma
The concept of the ischemic penumbra is central to acute treatment: surrounding the irreversibly infarcted core is a zone of reversibly ischemic tissue that can be salvaged if blood flow is restored promptly.
(Adams and Victor's Principles of Neurology, 12th Edition)

Causes of Ischemic Stroke

Atherosclerotic thrombosis - the most common cause in middle-aged and elderly patients; plaques form at vessel bifurcations (carotid sinus, basilar artery)
Cardioembolic stroke - major sources include:
  • Atrial fibrillation (most common; risk quantified by CHA₂DS₂-VASc score)
  • Mural thrombus post-myocardial infarction
  • Valvular heart disease (mitral stenosis, prosthetic valves, endocarditis vegetations)
  • Cardiac myxoma
Artery-to-artery embolism - ulcerated atheromatous plaque in the carotid or vertebral arteries; aortic arch plaques >4 mm are found in ~38% of cryptogenic stroke patients
Small vessel (lacunar) disease - hypertension causes lipohyalinosis and fibrinoid necrosis of penetrating arteries, producing discrete small infarcts in the basal ganglia, internal capsule, thalamus, and pons
Cryptogenic stroke - up to 15-30% have no identified cause; long-term cardiac monitoring (implanted loop recorders) reveals occult atrial fibrillation in up to 15%
(Adams and Victor's, 12th Ed.)

Transient Ischemic Attack (TIA)

A TIA is defined as transient neurologic dysfunction caused by focal ischemia of brain, spinal cord, or retina, without acute infarction. Classically resolves within 24 hours, but modern imaging shows infarction may persist. The risk of subsequent stroke after TIA is:
  • Up to 10% at 2 days
  • Up to 15% at 90 days
Conditions confused with TIA/stroke: migraine, Todd's paralysis (postictal paresis), hypoglycemia, subdural hematoma, brain tumor, conversion disorder.
(Frameworks for Internal Medicine)

II. Hemorrhagic Stroke

Intracerebral Hemorrhage (ICH)

Bleeding directly into the brain parenchyma. Causes injury by mechanical compression of brain tissue and local toxicity from blood breakdown products.
Causes:
  • Chronic hypertension (most common - causes Charcot-Bouchard microaneurysms in penetrating arteries)
  • Coagulopathies (endogenous or drug-related, e.g., warfarin) - notably, novel oral anticoagulants (NOACs) carry a significantly lower ICH risk than warfarin
  • Cerebral amyloid angiopathy (in elderly)
  • Vascular malformations (AVMs, cavernous malformations)
  • Secondary hemorrhage into ischemic infarct
Typical locations in hypertensive ICH: putamen, thalamus, cerebellum, pons, subcortical white matter

Subarachnoid Hemorrhage (SAH)

Bleeding into the subarachnoid space. Onset is almost instantaneous - classically described as the "worst headache of my life" (thunderclap headache).
Causes:
  • Ruptured saccular (berry) aneurysm at the Circle of Willis (~85%)
  • Arteriovenous malformations (AVMs)
  • Trauma
Diagnosis: Non-contrast CT is highly sensitive in the first 12-24 hours. If CT is negative but SAH is suspected, lumbar puncture should be performed to look for xanthochromia.

III. Other Cerebrovascular Diseases

Cerebral Venous Sinus Thrombosis

Thrombosis of dural venous sinuses, may be secondary to infections (ear, paranasal sinus), meningitis, puerperium, or hematologic disorders (polycythemia, sickle cell disease, protein C/S deficiency).

Arteritis/Vasculitis

  • Infective: meningovascular syphilis, TB meningitis, fungal (mucormycosis)
  • Autoimmune: polyarteritis nodosa, SLE, temporal/giant cell arteritis, Granulomatosis with polyangiitis, Takayasu disease

Cerebral Amyloid Angiopathy (CAA)

Amyloid deposition in cortical and leptomeningeal vessel walls; major cause of lobar ICH in the elderly.

CADASIL and Inherited Cerebrovascular Disorders

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL); caused by NOTCH3 mutations.

Moyamoya Disease

Progressive stenosis/occlusion of the terminal internal carotid arteries with collateral "puff of smoke" vessel formation; important cause of childhood stroke.

IV. Causes by Age Group

(Adams and Victor's Principles of Neurology, 12th Ed.)
AgeKey Causes
PerinatalCardiorespiratory failure, periventricular infarcts, matrix hemorrhages
Infancy/ChildhoodCongenital heart disease, Moyamoya, bacterial endocarditis, sickle cell anemia, MELAS, homocystinuria
Adolescence/Young adultPregnancy, OCP-related, migraine, vascular malformations, arteritis, arterial dissections, antiphospholipid syndrome
Middle ageAtherosclerotic thrombosis, cardiogenic embolism, hypertensive ICH, ruptured aneurysm, dissection, fibromuscular dysplasia
Late adult lifeAtherosclerotic thrombosis, lacunar stroke, brain hemorrhage (multiple causes), vascular dementia, Binswanger disease

V. Clinical Approach to Stroke

A structured approach involves four steps (Adams and Victor's):
  1. Determine if the event is a stroke (vs. migraine, seizure, syncope, hypoglycemia)
  2. Determine the pathophysiology (ischemic vs. hemorrhagic; embolic, thrombotic, or lacunar)
  3. Initiate acute treatment if appropriate (tPA or endovascular thrombectomy for ischemic; neurosurgical intervention or BP management for hemorrhagic)
  4. Plan secondary prevention (antiplatelet therapy, anticoagulation, carotid revascularization, risk factor control)

Imaging

  • Non-contrast CT: First-line; highly sensitive for acute ICH; less sensitive for early ischemic stroke (especially posterior fossa)
  • MRI (DWI): Much higher sensitivity for ischemic stroke within hours of onset; preferred where available

VI. Risk Factors

Chronic hypertension is the single strongest risk factor - present in most stroke patients (SBP ≥140 or DBP ≥90 mmHg). Other major risk factors include:
  • Diabetes mellitus
  • Atrial fibrillation and other cardiac dysrhythmias
  • Dyslipidemia
  • Cigarette smoking
  • Physical inactivity
  • Poor nutrition
  • Family history/genetics
  • Chronic kidney disease
(Frameworks for Internal Medicine)

VII. Stroke in Children

Pediatric stroke incidence is approximately 2 cases per 100,000 children per year. The aetiology differs fundamentally from adults - atherosclerosis, cardioembolic disease, and small vessel disease account for only ~10% of cases. Associated factors include congenital heart disease, anaemias, prothrombotic disorders (protein C and S deficiencies), hyperhomocysteinaemia, lipid abnormalities, recent infections, and mitochondrial respiratory chain disorders. Long-term prognosis is poor - some degree of dependency occurs in 60% of affected children. Brain MRI is the recommended imaging modality, performed as soon as possible. (Grainger & Allison's Diagnostic Radiology)

VIII. Vascular Dementia

Multifocal or diffuse cerebrovascular disease is an important cause of dementia in the elderly. Binswanger disease (subcortical arteriosclerotic leukoencephalopathy) results from diffuse ischemic damage to deep white matter from small vessel hypertensive disease, presenting as progressive cognitive decline with gait disturbance and urinary incontinence.

Summary Diagram

Mechanisms of hemorrhagic and ischemic stroke
Figure: Classification of stroke into hemorrhagic and ischemic types (Frameworks for Internal Medicine, p. 575)
Key Sources:
  • Adams and Victor's Principles of Neurology, 12th Edition, Chapter 33 - Stroke and Cerebrovascular Diseases
  • Frameworks for Internal Medicine - Chapter 43 (Stroke)
  • Grainger & Allison's Diagnostic Radiology - Cerebrovascular Disease and Stroke
  • Bradley and Daroff's Neurology in Clinical Practice - Stroke and Cerebral Vascular Disease
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