Neurosurgery notes/Vascular/Occlusive disease/Cerebral small vessel disease

Cerebral small vessel disease

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Definition

  • Is an umbrella term for lesions in the brain attributed to pathology of small arteries, arterioles, capillaries, venules, or small veins.

Aka

  • Cerebral microangiopathy
  • Leukoaraiosis
    • A radiological descriptor applied to white matter hypodensities on CT and high signal changes on T2-weighted MRI of presumed vascular origin

Numbers

  • More common with increasing age
    • The prevalence of white matter lesions in the general population is reported to be between 39 to 96%

Differential diagnosis

  • Arteriolosclerosis (age-related and vascular risk factor-related small vessel disease):
    • Binswanger disease
      • notion image
  • Cerebral amyloid angiopathy (sporadic or hereditary)
  • Inherited/genetic small vessel diseases other than cerebral amyloid angiopathy, such as
    • CADASIL
      • notion image
    • CARASIL
    • MELAS
    • Fabry disease
    • Retinal vasculopathy with cerebral leukoencephalopathy
    • COL4A1 brain small-vessel disease
  • Inflammatory and immunologically mediated small vessel diseases (CNS vasculitis)
  • Venous collagenosis
  • Other small vessel diseases, such as
  • Post-radiation angiopathy
  • Non-amyloid microvessel degeneration in Alzheimer disease

Pathology

  • Juxtaventricular white matter changes
    • (<3 mm from the ventricular surface),
    • Eg ependymitis granularis, are not related to small vessel disease, but rather represent cerebrospinal fluid leak due to disruption of the ependyma
  • Periventricular white matter lesions
    • Periventricular defined as 3-13 mm from the ventricular surface
    • This area is haemodynamically determined
      • This region is a vascular border zone vascularised by non-collateralising ventriculofugal vessels arising from subependymal arteries --> prone to local and systemic decrease in cerebral blood flow --> watershed infarcts especially when located along the posterior horns and it is correlated with carotid artery stenosis 8.
    • A predictor of watershed infarcts
  • Deep and subcortical white matter lesions
    • Deep white matter changes (>13 mm from the ventricular surface, >4 mm from the corticomedullary junction)
    • Due to lipohyalinosis (small vessel disease), i.e. incomplete arteriosclerosis.
    • A predictor of lacunar infarcts.