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Spino-Cerebello-Cerebral Degeneration with Amyloid Plaques (Gerstmann, Sträussler, Scheinker Syndrome)

Published online by Cambridge University Press:  18 September 2015

Clarisse L. Dolman  and
Lyle L. Daly
Clarisse L. Dolman*
Affiliation:
Department of Pathology, Vancouver General Hospital and the University of British Columbia, Vancouver, B.C
Lyle L. Daly
Affiliation:
Department of Pathology, Vancouver General Hospital and the University of British Columbia, Vancouver, B.C
*
Department of Pathology, Vancouver General Hospital, 855 West 12th Avenue, Vancouver, B.C. V5Z 1M9
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Summary:

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We report the case of a man who died at age 40 after a 9 year illness resembling a hereditary spinocerebellar degeneration, but accompanied also by brain stem signs and dementia. Autopsy disclosed lesions of combined spinocerebellar and ponto-cerebellar degeneration, but also damage to many brain stem nuclei. Prominent amyloid plaques disseminated through the brain indicated a relationship to the Kuru-Creutzfeldt-Jakob group of diseases. Transmission experiments are underway at the National Institutes of Health.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 9 , Issue 4 , November 1982 , pp. 439 - 442
Copyright
Copyright © Canadian Neurological Sciences Federation 1982

References

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