Almås T, Vedeler C, Moen G, Mørk S
Nevrologisk avdeling, Haukeland Sykehus, Bergen.
Tidsskr Nor Laegeforen. 1998 Aug 30;118(20):3123-6.
Cerebral amyloid angiopathy affects the cerebral vasculature selectively, and there is no systemic amyloidosis. Amyloid is deposited in small and medium-sized vessels of the cortex and leptomeninges. Cerebral amyloid angiopathy is a common cause of spontaneous lobar haemorrhage in elderly patients. However, cerebral amyloid angiopathy may have atypical clinical and radiological presentations. We report on five patients (three males and two females, aged 43-77 years) with histologically verified cerebral amyloid angiopathy. One patient experienced an acute headache attack and classical lobar haemorrhage. The other patients had various neurological symptoms and signs, such as seizure, disturbed vision, pareses, aphasia, and dementia that were initially diagnosed as cerebral infarction or tumour. Two patients with cerebral amyloid angiopathy and granulomatous angiitis responded to immunosuppressive treatment.
脑淀粉样血管病选择性地累及脑血管系统，不存在系统性淀粉样变性。淀粉样物质沉积于皮质和软脑膜的中小血管。脑淀粉样血管病是老年患者自发性脑叶出血的常见原因。然而，脑淀粉样血管病可能有非典型的临床和影像学表现。我们报告了5例经组织学证实为脑淀粉样血管病的患者（3例男性，2例女性，年龄43 - 77岁）。1例患者经历了急性头痛发作和典型的脑叶出血。其他患者有各种神经症状和体征，如癫痫发作、视力障碍、轻瘫、失语和痴呆，最初被诊断为脑梗死或肿瘤。2例脑淀粉样血管病合并肉芽肿性血管炎的患者对免疫抑制治疗有反应。
