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Table 1:

Etiopathogenic classification of cerebral small vessel diseases

Classification
Type 1: HAFibrinoid necrosis, lipohyalinosis, microatheroma, microaneurysms
Type 2: Sporadic and hereditary CAA
Type 3: Inherited or genetic SVD (distinct from CAA)For example, CADASIL, CARASIL, MELAS, Fabry disease, COL4A1 mutations, and so forth
Type 4: Inflammatory- and immunologically-mediated SVDExamples: Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, Henoch-Schönlein purpura, cryoglobulinemic vasculitis, cutaneous leukocytoclastic angiitis, primary angiitis of the CNS, Sjögren syndrome, rheumatoid vasculitis, scleroderma, dermatomyositis, and so forth
Type 5: Venous collagenosis
Type 6: Other SVDExamples: postradiation angiopathy and nonamyloid microvessel degeneration in Alzheimer disease
  • Note:—MELAS indicates Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes; CARASIL, cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy.