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Article

Gerstmann-Sträussler-Scheinker disease in an Alsatian family: clinical and genetic studies.

Journal of neurology, neurosurgery, and psychiatry - 1 Mar 1992

Tranchant C, Doh-ura K, Warter J M, Steinmetz G, Chevalier Y, Hanauer A, Kitamoto T, Tateishi J

Abstract excerpt

The clinical progression of Gerstmann-Sträussler-Scheinker disease in a family of Alsatian origin is reported. The age of onset and the duration of evolution were variable. The clinical picture became more complex over the generations: in the first generations, isolated dementia and in later generations a triad of pyramidal, pseudobulbar syndromes and dementia associated with spinal cord and cerebellar features....

Topics

Adult
Chromosome Aberrations
Chromosome Disorders
Codon
Female
France
Genes, Dominant
Gerstmann-Straussler-Scheinker Disease
Humans
Male
Middle Aged

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Mutation

Pedigree

PrPSc Proteins

Prions

Source

Open in PubMed Open full text

Identifiers

PMID: 1348783
DOI: 10.1136/jnnp.55.3.185
PMCID: PMC1014721

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 29 Aug 2026. Prepared record publication-1348783-2026-08-29.1. Manifest publication-graph-cohort-2026-08-29.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 29 Aug 2026.

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