Rauschka Helmut, Retzl Johann, Baumhackl Ulf, Bankl Hans Christian, Salomonowitz Erich, Schmidbauer Manfred
Department of Neurology, Municipal Hospital Lainz, Wolkersbergenstrasse 1, A-1130 Vienna, Austria.
J Neurol Sci. 2003 Apr 15;208(1-2):101-4. doi: 10.1016/s0022-510x(02)00395-7.
A previously healthy 69-year-old man developed a progressive neurological illness with bulbar signs and ataxic paraparesis. Repeated MRI examinations revealed a large space occupying lesion in the lower brain stem with patchy contrast enhancement. MRI angiography was unremarkable and CSF had normal cell count but raised protein content. A brainstem tumor was suspected and a course of intravenous glucocorticosteroids was started. No improvement occurred and the patient died of pneumonia 11 weeks after onset. Neuropathology revealed confluent areas of complete or incomplete necrosis with marked edema in the lower brainstem. Predominantly venous meningeal vessels of the brainstem showed extensive fibromuscular thickening of all layers with luminal narrowing. In addition, intramural mononuclear infiltration was found. With the exception of localisation, this case exhibits all pathologic features of subacute diencephalic angioencephalopathy (SDAE), a rare fatal disease of unknown aetiology. In addition, the clinical features of typical age, male sex, disease duration and raised CSF proteins are shared. A common disease entity is suggested and the pathogenetic relevance of inflammation and venous outflow obstruction is discussed.
一名69岁既往健康的男性出现了一种进行性神经系统疾病，伴有延髓体征和共济失调性截瘫。多次MRI检查显示脑桥下部有一个较大的占位性病变，有斑片状强化。MRI血管造影无异常，脑脊液细胞计数正常但蛋白含量升高。怀疑为脑干肿瘤，开始了静脉注射糖皮质激素治疗。病情无改善，患者在发病11周后死于肺炎。神经病理学显示脑桥下部有融合的完全或不完全坏死区域，并伴有明显水肿。脑干主要的静脉性脑膜血管显示所有层均有广泛的纤维肌性增厚，管腔狭窄。此外，还发现壁内单核细胞浸润。除了病变部位外，该病例表现出亚急性间脑血管性脑病（SDAE）的所有病理特征，这是一种病因不明的罕见致命疾病。此外，还具有典型的年龄、男性性别、病程和脑脊液蛋白升高的临床特征。提示存在一种常见的疾病实体，并讨论了炎症和静脉流出道梗阻的发病机制相关性。
