LeWitt P A, Forno L S, Brant-Zawadzki M
Neurology. 1983 Jan;33(1):39-44. doi: 10.1212/wnl.33.1.39.
Neoplastic angioendotheliosis (NA) of the CNS is usually characterized by systemic vascular disease and a rapidly fatal course. We report a 52-year-old woman with dementia, spasticity, and sensory deficits developing insidiously over a year. Diagnostic findings included small CT lucencies in the brain and angiographic irregularities of medium-sized arteries (resembling cerebral arteritis). Brain biopsy revealed numerous small infarcts as well as pleomorphic, highly malignant tumor cells within cerebral meningeal vessels. Without treatment, she experienced only slight increase of dementia before death from pneumonia. At autopsy, there was almost complete regression of the intravascular cerebral tumor. The clinical course was unusual for the length of illness and the radiographic picture of cerebral vasculitis. Clinical features often present with NA--such as strokelike events, elevated sedimentation rate, renal impairment, and fever--were notably absent.
中枢神经系统肿瘤性血管内皮增生症（NA）通常以全身性血管疾病和快速致命病程为特征。我们报告了一名52岁女性，她在一年多时间里逐渐出现痴呆、痉挛和感觉障碍。诊断结果包括脑部CT低密度影以及中等大小动脉的血管造影不规则（类似脑动脉炎）。脑活检显示大量小梗死灶以及脑脑膜血管内多形性、高度恶性的肿瘤细胞。未经治疗，她在因肺炎死亡前痴呆仅略有加重。尸检时，脑内血管肿瘤几乎完全消退。该临床病程在疾病持续时间和脑血管炎影像学表现方面都不寻常。NA常见的临床特征，如类卒中事件、血沉升高、肾功能损害和发热，均未出现。
