Early-onset dementia and extrapyramidal disease: Clinicopathological variant of Gerstmann-Straussler-Scheinker or Alzheimer's disease?

J. Hart, B. Gordon

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

A case of progressive dementia and extrapyramidal signs beginning at age 29, with a ten year course until death, is presented. Necropsy examination showed an assortment of plaque types (including striatal plaques), neurofibrillary tangles, granulovacuolar degeneration, and depigmentation of the substantia nigra and locus ceruleus. This case had pathological features found in both Gerstmann-Straussler-Scheinker disease and in Alzheimer's disease. While somewhat similar to several other cases with features of both diseases, it differs in the presence of dystonia and striatal plaques. Although such cases may be difficult to categorise at present, they must be considered in the differential diagnosis of early onset dementia.

Original languageEnglish (US)
Pages (from-to)932-934
Number of pages3
JournalJournal of Neurology, Neurosurgery and Psychiatry
Volume53
Issue number11
DOIs
StatePublished - 1990

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Gerstmann-Straussler-Scheinker Disease
Corpus Striatum
Dementia
Alzheimer Disease
Neurofibrillary Tangles
Locus Coeruleus
Dystonia
Substantia Nigra
Differential Diagnosis

ASJC Scopus subject areas

  • Public Health, Environmental and Occupational Health
  • Psychiatry and Mental health
  • Neuroscience(all)
  • Neuropsychology and Physiological Psychology

Cite this

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title = "Early-onset dementia and extrapyramidal disease: Clinicopathological variant of Gerstmann-Straussler-Scheinker or Alzheimer's disease?",
abstract = "A case of progressive dementia and extrapyramidal signs beginning at age 29, with a ten year course until death, is presented. Necropsy examination showed an assortment of plaque types (including striatal plaques), neurofibrillary tangles, granulovacuolar degeneration, and depigmentation of the substantia nigra and locus ceruleus. This case had pathological features found in both Gerstmann-Straussler-Scheinker disease and in Alzheimer's disease. While somewhat similar to several other cases with features of both diseases, it differs in the presence of dystonia and striatal plaques. Although such cases may be difficult to categorise at present, they must be considered in the differential diagnosis of early onset dementia.",
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AB - A case of progressive dementia and extrapyramidal signs beginning at age 29, with a ten year course until death, is presented. Necropsy examination showed an assortment of plaque types (including striatal plaques), neurofibrillary tangles, granulovacuolar degeneration, and depigmentation of the substantia nigra and locus ceruleus. This case had pathological features found in both Gerstmann-Straussler-Scheinker disease and in Alzheimer's disease. While somewhat similar to several other cases with features of both diseases, it differs in the presence of dystonia and striatal plaques. Although such cases may be difficult to categorise at present, they must be considered in the differential diagnosis of early onset dementia.

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