Spacek J, Nozicka Z
Department of Pathology, Charles University Faculty of Medicine, Hradec Kralove, Czech Republic.
Hum Pathol. 1994 Dec;25(12):1366-70. doi: 10.1016/0046-8177(94)90099-x.
We describe what we believe to be the fifth case of a degenerative condition of the brain characterized by unusual intracytoplasmic inclusions in neocortical astrocytes, and we review four previous reports of what appears to be the same condition. Whereas these previous cases were characterized by prolonged clinical mental and psychomotor retardation, our case describes a rapid onset in a previously fit and mentally able patient in whom the astrocytic inclusions showed a close association with fibrohyaline vascular degeneration and changes resembling those of Alzheimer's disease. The inclusions, which were most frequent in the second to fourth layers of the frontal, temporal, and occipital cortices but absent from subcortical regions, consisted of large, irregular hyaline bodies surrounding the nucleus and extending into the proximal parts of cell processes. Ultrastructurally they consisted of free ribosomes in a granular and filamentous matrix. They were not bound by a membrane. Lipofuscin granules were associated with them. It is suggested that the inclusions might result from a disturbance of protein metabolism in protoplasmic astrocytes, but their true significance is unknown.
我们描述了我们认为是第五例以新皮质星形胶质细胞内出现异常胞浆内包涵体为特征的脑部退行性疾病，并回顾了之前关于似乎是同一病症的四份报告。之前的这些病例以长期的临床精神和精神运动发育迟缓为特征，而我们的病例描述的是一名此前健康且智力正常的患者发病迅速，其星形胶质细胞包涵体与纤维透明血管变性以及类似阿尔茨海默病的变化密切相关。包涵体在额叶、颞叶和枕叶皮质的第二至第四层最为常见，但皮质下区域没有，由围绕细胞核并延伸至细胞突起近端部分的大的不规则透明体组成。超微结构上，它们由颗粒状和丝状基质中的游离核糖体组成。它们没有被膜包裹。脂褐素颗粒与它们相关。提示这些包涵体可能是由原浆性星形胶质细胞中蛋白质代谢紊乱导致的，但其真正意义尚不清楚。
