原發性中樞神經系統血管炎：一病例報告及文獻回顧

Primary Angiitis of Central Nervous System - A Case Report and Literature Review

原發性中樞神經血管炎是一種少見的疾病。本篇報告描述一位34歲女性發生了右側肢體無力，眼皮下垂等的症狀，但在中風的傳統治療下仍不斷惡化。在一連串檢查，包括心臟超音波，antithrombin III, protein C, protein S, antiphospholipid antibodies及lups anticoagulant等等，排除其他造成早發性中風的可能性之後，血管攝影檢查發現兩側腦血管有多處不規則狹窄的情形。在高度懷疑是血管炎的病變下，病患接受合併Corticosteroid與Cyclophosphamide的脈衝治療後，復元情況良好。我們藉由此一病例與文獻回顧來討論此一少見的中樞神經性血管炎。

Primary angiitis of central nervous system (PACNS) is a rare form of vasculitis. We described a 34-year-old women with initial presentation of acute stroke symptoms. Her neurological deficits were in progression under the treatment of ischemic stroke. The possible etiologies in young stroke including congenital or acquired heart disease, hematological disorder, coagulopathies, congenital metabolic anormalies and CNS infection, were all excluded after work-up. Angiography showed irregular narrowing of the lumen of bilateral PCA and SCA and arteritis was highly suspected by the image study. She was treated with the combination pulse therapy of corticosteroid and cyclophosphamide successfully except some neurological sequels left. Here, we review and discuss the diagnosis and treatment of PACNS.