Schmidbauer M, Lassmann J, Pilz P, Vass K, Budka H
Neurologisches Institut der Universität, Wien, Austria.
J Neurol. 1992 Aug;239(7):379-81. doi: 10.1007/BF00812154.
A previously healthy 58-year-old man developed neurological illness with progressive dementia, hallucinations, central motor and vegetative impairment which led to death in 14 weeks. Autopsy revealed lesions in a symmetrical centrencephalic distribution. Inner cerebral veins and arteries were surrounded by extravasation of plasma and perivascular haemorrhage and were thickened by fibrous scarring and muscle fibre proliferation. Necrotized blood vessels were also found. The parenchyma was damaged by incomplete to complete necrosis. The age and sex of the patient, the progressive clinical course, the increase of cerebrospinal fluid protein, and the histopathology of the lesion show some similarities to angiodysgenetic necrotizing encephalopathy and spinal Foix-Alajouanine disease.
一名58岁既往健康的男性出现神经系统疾病，伴有进行性痴呆、幻觉、中枢运动及自主神经功能障碍，于14周后死亡。尸检发现病变呈对称性中央脑区分布。大脑内静脉和动脉周围有血浆外渗和血管周围出血，且因纤维瘢痕形成和肌纤维增生而增厚。还发现了坏死血管。实质受到不完全至完全坏死的损害。患者的年龄、性别、进行性临床病程、脑脊液蛋白升高以及病变的组织病理学与血管发育异常性坏死性脑病和脊髓福-阿二氏病有一些相似之处。
