Familial clustering of the ataxic form of Creutzfeldt-Jakob disease with Hirano bodies.
- 1 March 1985
- journal article
- research article
- Published by BMJ in Journal of Neurology, Neurosurgery & Psychiatry
- Vol. 48 (3) , 234-238
- https://doi.org/10.1136/jnnp.48.3.234
Abstract
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.Keywords
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