Cerebellar plaques in familial Alzheimer's disease (Gerstmann-Sträussler-Scheinker variant?)

B. Azzarelli, J. Muller, Bernardino Ghetti, M. Dyken, P. M. Conneally

Research output: Contribution to journalArticle

50 Citations (Scopus)

Abstract

A large kindred, with two brothers coming to autopsy, of a syndrome consisting of ataxia, dementia, and some Parkinsonian features is reported; inheritance appears to be autosomal dominant. Neuropathologically, there were plaques and neurofibrillary tangles in the cerebral cortex as well as some in the basal ganglia, particularly reminiscent of the plaques seen in Kuru; there was only minimal spinal cord disease (pyramidal tract field). The problems of classifying this condition - Alzheimer's disease with cerebellar involvement or other entities, such as the Gerstmann-Sträussler-Scheinker condition (1936), especially now that transmission to animals in the latter has been reported - are discussed. Some relevant theoretical considerations derived from animal work, particulary in scrapie, are also reviewed.

Original languageEnglish
Pages (from-to)235-246
Number of pages12
JournalActa Neuropathologica
Volume65
Issue number3-4
DOIs
StatePublished - Sep 1985

Fingerprint

Alzheimer Disease
Kuru
Scrapie
Pyramidal Tracts
Neurofibrillary Tangles
Spinal Cord Diseases
Ataxia
Basal Ganglia
Cerebral Cortex
Dementia
Autopsy

Keywords

  • Alzheimer disease
  • Cerebellar plaques
  • Gerstmann-Sträussler-Scheinker disease
  • Hereditary ataxia
  • Neurofibrillary tangles

ASJC Scopus subject areas

  • Neuroscience(all)
  • Pathology and Forensic Medicine
  • Clinical Neurology

Cite this

Cerebellar plaques in familial Alzheimer's disease (Gerstmann-Sträussler-Scheinker variant?). / Azzarelli, B.; Muller, J.; Ghetti, Bernardino; Dyken, M.; Conneally, P. M.

In: Acta Neuropathologica, Vol. 65, No. 3-4, 09.1985, p. 235-246.

Research output: Contribution to journalArticle

Azzarelli, B. ; Muller, J. ; Ghetti, Bernardino ; Dyken, M. ; Conneally, P. M. / Cerebellar plaques in familial Alzheimer's disease (Gerstmann-Sträussler-Scheinker variant?). In: Acta Neuropathologica. 1985 ; Vol. 65, No. 3-4. pp. 235-246.
@article{7e967a5083e64df8843950b5230f13ef,
title = "Cerebellar plaques in familial Alzheimer's disease (Gerstmann-Str{\"a}ussler-Scheinker variant?)",
abstract = "A large kindred, with two brothers coming to autopsy, of a syndrome consisting of ataxia, dementia, and some Parkinsonian features is reported; inheritance appears to be autosomal dominant. Neuropathologically, there were plaques and neurofibrillary tangles in the cerebral cortex as well as some in the basal ganglia, particularly reminiscent of the plaques seen in Kuru; there was only minimal spinal cord disease (pyramidal tract field). The problems of classifying this condition - Alzheimer's disease with cerebellar involvement or other entities, such as the Gerstmann-Str{\"a}ussler-Scheinker condition (1936), especially now that transmission to animals in the latter has been reported - are discussed. Some relevant theoretical considerations derived from animal work, particulary in scrapie, are also reviewed.",
keywords = "Alzheimer disease, Cerebellar plaques, Gerstmann-Str{\"a}ussler-Scheinker disease, Hereditary ataxia, Neurofibrillary tangles",
author = "B. Azzarelli and J. Muller and Bernardino Ghetti and M. Dyken and Conneally, {P. M.}",
year = "1985",
month = "9",
doi = "10.1007/BF00687003",
language = "English",
volume = "65",
pages = "235--246",
journal = "Acta Neuropathologica",
issn = "0001-6322",
publisher = "Springer Verlag",
number = "3-4",

}

TY - JOUR

T1 - Cerebellar plaques in familial Alzheimer's disease (Gerstmann-Sträussler-Scheinker variant?)

AU - Azzarelli, B.

AU - Muller, J.

AU - Ghetti, Bernardino

AU - Dyken, M.

AU - Conneally, P. M.

PY - 1985/9

Y1 - 1985/9

N2 - A large kindred, with two brothers coming to autopsy, of a syndrome consisting of ataxia, dementia, and some Parkinsonian features is reported; inheritance appears to be autosomal dominant. Neuropathologically, there were plaques and neurofibrillary tangles in the cerebral cortex as well as some in the basal ganglia, particularly reminiscent of the plaques seen in Kuru; there was only minimal spinal cord disease (pyramidal tract field). The problems of classifying this condition - Alzheimer's disease with cerebellar involvement or other entities, such as the Gerstmann-Sträussler-Scheinker condition (1936), especially now that transmission to animals in the latter has been reported - are discussed. Some relevant theoretical considerations derived from animal work, particulary in scrapie, are also reviewed.

AB - A large kindred, with two brothers coming to autopsy, of a syndrome consisting of ataxia, dementia, and some Parkinsonian features is reported; inheritance appears to be autosomal dominant. Neuropathologically, there were plaques and neurofibrillary tangles in the cerebral cortex as well as some in the basal ganglia, particularly reminiscent of the plaques seen in Kuru; there was only minimal spinal cord disease (pyramidal tract field). The problems of classifying this condition - Alzheimer's disease with cerebellar involvement or other entities, such as the Gerstmann-Sträussler-Scheinker condition (1936), especially now that transmission to animals in the latter has been reported - are discussed. Some relevant theoretical considerations derived from animal work, particulary in scrapie, are also reviewed.

KW - Alzheimer disease

KW - Cerebellar plaques

KW - Gerstmann-Sträussler-Scheinker disease

KW - Hereditary ataxia

KW - Neurofibrillary tangles

UR - http://www.scopus.com/inward/record.url?scp=0021940757&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0021940757&partnerID=8YFLogxK

U2 - 10.1007/BF00687003

DO - 10.1007/BF00687003

M3 - Article

VL - 65

SP - 235

EP - 246

JO - Acta Neuropathologica

JF - Acta Neuropathologica

SN - 0001-6322

IS - 3-4

ER -