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. 1985 Mar;48(3):234–238. doi: 10.1136/jnnp.48.3.234

Familial clustering of the ataxic form of Creutzfeldt-Jakob disease with Hirano bodies.

L Cartier, S Gálvez, D C Gajdusek
PMCID: PMC1028256  PMID: 2984334

Abstract

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.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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