Mühleisen H, Gehrmann J, Meyermann R
Department of Pathology, University Hospital, Zürich, Switzerland.
Neuropathol Appl Neurobiol. 1995 Dec;21(6):505-17. doi: 10.1111/j.1365-2990.1995.tb01097.x.
Creutzfeldt-Jakob disease (CJD) is characterized by a loss of neurons accompanied by astrogliosis and spongiform changes in the neuropil. It has been recognized that reactive microglia occur in CJD but little is known about the regional distribution and extent of the microglial activation. We have, therefore, examined six brains from cases of sporadic CJD by immunohistochemical labelling of grey and white matter microglia from frontal, parietal, temporal, and occipital lobes, striatum, thalamus, cerebellum and brain stem with RCA-1, LCA, CD68, HLA-DR, and HAM56. Microglial activation occurred in the grey matter where astrogliosis and prion protein (PrP) deposits were prominent. Processes of activated microglia surrounded the outer rim of spongy vacuoles. A diffuse microglial response was seen in the white matter that was immunophenotypically different from grey matter. Double-labelling with microglial markers and anti-PrP showed that activated microglia did not contain PrP-immunoreactivity. Therefore a primary role of microglia in PrP processing seems unlikely. Activated microglia may contribute to neuronal damage in CJD due to their cytotoxic potential.
克雅氏病（CJD）的特征是神经元丧失，伴有星形胶质细胞增生和神经毡的海绵状改变。人们已经认识到，反应性小胶质细胞在CJD中出现，但对小胶质细胞活化的区域分布和程度了解甚少。因此，我们通过用RCA-1、LCA、CD68、HLA-DR和HAM56对额叶、顶叶、颞叶和枕叶、纹状体、丘脑、小脑和脑干的灰质和白质小胶质细胞进行免疫组织化学标记，检查了6例散发性CJD病例的大脑。小胶质细胞活化发生在星形胶质细胞增生和朊病毒蛋白（PrP）沉积突出的灰质中。活化小胶质细胞的突起围绕着海绵状空泡的外缘。在白质中可见弥漫性小胶质细胞反应，其免疫表型与灰质不同。用小胶质细胞标记物和抗PrP进行双重标记显示，活化的小胶质细胞不含有PrP免疫反应性。因此，小胶质细胞在PrP加工中的主要作用似乎不太可能。活化的小胶质细胞可能因其细胞毒性潜力而导致CJD中的神经元损伤。
