Cartier L, Gálvez S, Gajdusek D C
J Neurol Neurosurg Psychiatry. 1985 Mar;48(3):234-8. doi: 10.1136/jnnp.48.3.234.
A family cluster of the ataxic form of Creutzfeldt-Jakob disease with one probable and two autopsy proven cases that occurred in a single generation between 1974 and 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.
报告了1974年至1982年间在一代人中发生的1例可能病例和2例经尸检证实的克雅氏病共济失调型家族聚集病例。这些病例的临床特征与库鲁病患者的特征极为相似，明显的躯干共济失调与小脑皮质尤其是蚓部的严重破坏之间存在较好的相关性。在病理上，典型的可传播性克雅氏病中罕见的明显海马受累，以及海马角出现 Hirano 小体而无特异性阿尔茨海默病老年改变，都是值得注意的特征。
