Gerstmann-Sträussler-Scheinker disease: Autopsy study of a familial case

Postmortem neuropathological findings in a patient with biopsy‐proved familial Gerstmann‐Sträussler‐Scheinker disease of eight years' duration included severe spongy change in the neocortex, extensive and often large amyloid deposits throughout the cerebral hemispheres and cerebellum, and sever...

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Veröffentlicht in:	Annals of neurology 1986-10, Vol.20 (4), p.540-543
Hauptverfasser:	Vinters, Harry V., Hudson, Arthur J., Kaufmann, John C. E.
Format:	Artikel
Sprache:	eng
Schlagworte:	Biological and medical sciences Central Nervous System - pathology Cerebellar Ataxia - genetics Cerebellar Ataxia - pathology Dementia - genetics Dementia - pathology Female Human viral diseases Humans Infectious diseases Medical sciences Middle Aged Reflex, Abnormal - genetics Reflex, Abnormal - pathology Syndrome Viral diseases Viral diseases of the nervous system
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container_title	Annals of neurology
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creator	Vinters, Harry V. Hudson, Arthur J. Kaufmann, John C. E.
description	Postmortem neuropathological findings in a patient with biopsy‐proved familial Gerstmann‐Sträussler‐Scheinker disease of eight years' duration included severe spongy change in the neocortex, extensive and often large amyloid deposits throughout the cerebral hemispheres and cerebellum, and severe astrocytic gliosis throughout all areas of gray and white matter within the brain. The degree of cortical spongy change was much greater than that in relatives who died with a similar clinical history, indicating the phenotypic heterogeneity in this familial disorder.
doi_str_mv	10.1002/ana.410200418
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The degree of cortical spongy change was much greater than that in relatives who died with a similar clinical history, indicating the phenotypic heterogeneity in this familial disorder.</abstract><cop>Hoboken</cop><pub>Wiley Subscription Services, Inc., A Wiley Company</pub><pmid>3789669</pmid><doi>10.1002/ana.410200418</doi><tpages>4</tpages></addata></record>
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subjects	Biological and medical sciences Central Nervous System - pathology Cerebellar Ataxia - genetics Cerebellar Ataxia - pathology Dementia - genetics Dementia - pathology Female Human viral diseases Humans Infectious diseases Medical sciences Middle Aged Reflex, Abnormal - genetics Reflex, Abnormal - pathology Syndrome Viral diseases Viral diseases of the nervous system
title	Gerstmann-Sträussler-Scheinker disease: Autopsy study of a familial case
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