Pervichnyy ß-amiloid-assotsiirovannyy vaskulit tsentral'noy nervnoy sistemy


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Resumo

Amyloid beta-related CNS vasculitis is one of the most rear types of primary CNS vasculitis. Recurrent hemorrhagic and ischemic strokes, epilepsy, severe cognitive decline are referred to be the main features of the disease. Pathological findings often include vasculitic transmural, often granulomatous, inflammatory infiltrates along with hemorrhages and amyloid-beta deposits seen in the meninges and in the brain. We describe the clinical course, radiologic and pathologic findings of a 33-year-old man with amyloid beta-related CNS vasculitis manifested in peripheral right facial nerve paralysis. During following three years, recurrent debilitating hemorrhagic, ischemic strokes and epilepsy were observed. Brain and meninges biopsy revealed granulomatous destructive angiitis with diffuse and local vessel amyloid-beta deposits in the meninges and brain tissue. ABRA was diagnosed and treatment with three PRED 1500 mg IV pulses followed by 75 mg orally and CYC 1400 mg IV every four weeks began. Such aggressive treatment suppressed inflammation and prevented the patient from radiologic progression.

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