FAMILIAL CLUSTERING OF THE ATAXIC FORM OF CREUTZFELDT-JAKOB DISEASE WITH HIRANO BODIES
FAMILIAL CLUSTERING OF THE ATAXIC FORM OF CREUTZFELDT-JAKOB DISEASE WITH HIRANO BODIES
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DOI：
10.1136/jnnp.48.3.234
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发表时间：
1985-01-01
影响因子：
11
通讯作者：
GAJDUSEK, DC
中科院分区：
文献类型：
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作者：
CARTIER, L;GALVEZ, S;GAJDUSEK, DC
据报道，克雅氏病共济失调型家族聚集，其中 1 例可能病例和 2 例尸检证实病例发生在 1974 年至 1982 年间的一代人中。这些病例的临床特征与库鲁病患者的临床特征非常相似，显着的躯干共济失调与小脑皮质（以蚓部最为明显）的严重破坏之间存在相当的相关性。在病理学上，典型的传染性克雅氏病中罕见的明显海马受累以及在阿蒙角中发现平野小体而没有特定的阿尔茨海默病老年变化是值得注意的特征。
A family cluster of the ataxic form of Creutzfeldt-Jakob disease with 1 probable and 2 autopsy proven cases that occurred in a single generation between 1974-1982 is reported. The clinical characteristics of the cases are closely similar to those of kuru patients, with a fair correlation between the prominent truncal ataxia and the intense devastation of the cerebellar cortex most marked in the vermis. Pathologically, the marked hippocampal involvement rarely seen in typical transmissible Creutzfeldt-Jakob disease and the finding of Hirano bodies in the Ammon''s horn without specific Alzheimer''s senile changes are noteworthy features.