Neuropathological study of cerebellar degeneration in prion disease

被引：14

作者：

Yang, Q ^{[1
]}

Hashizume, Y ^{[1
]}

Yoshida, M ^{[1
]}

Yang, Y ^{[1
]}

机构：

[1] Aichi Med Univ, Inst Med Sci, Nagakute, Aichi 4801195, Japan

来源：

NEUROPATHOLOGY | 1999年 / 19卷 / 01期

关键词：

cerebellum; Creutzfeldt-Jakob disease; Gerstmann-Straussler syndrome; neuropathology; prion protein;

D O I：

10.1046/j.1440-1789.1999.00212.x

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

In order to clarify the relationship between cerebellar degeneration and prion protein (PrP) accumulation, 17 cases of sporadic Creutzfeldt-Jakob disease (CJD) and three cases of Gerstmann-Straussler syndrome (GSS) were analyzed neuropathologically. Immunohistochemical staining for PrP showed synaptic-type deposits in all of the CJD cases, The punctate PrP stains in CJD patients were almost identical with those of synaptophysin, suggesting that PrP had accumulated in the synaptic structures. Synapses damage due to PrP accumulation seemed to develop prior to granule cell loss. PrP accumulation was inversely correlated with granule cell loss. The degree of atrophy in the molecular layer was compatible,vith granule cell loss. The nodulus showed severe PrP accumulation when compared with other sites in the cerebellum, However, in GSS cases, the loss of Purkinje cells and dentate nucleus neurons was obvious. Further, immunohistochemical staining for PrP demonstrated Kuru plaque-type and synaptic-type depositions in the molecular layer, the granular cell layer, and the dentate nucleus, which was different from CJD cases.

引用

页码：33 / 39

页数：7

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