Diseases

Gerstmann-Straussler-Scheinker Disease

An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal signs, and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration. (From Brain Pathol 1998 Jul;8(3):499-513; Brain Pathol 1995 Jan;5(1):61-75)

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal signs, and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration. (From Brain Pathol 1998 Jul;8(3):499-513; Brain Pathol 1995 Jan;5(1):61-75)

MeSH Record

Classification

Broader headings

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MeSH Record

Synonyms

15 entry terms
  • Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type
  • Gerstmann-Straussler Disease
  • Gerstmann-Straussler Inherited Spongiform Encephalopathy
  • Gerstmann-Straussler Syndrome
  • Gerstmann-Straussler-Scheinker Syndrome
  • Inherited Spongiform Encephalopathy, Gerstmann-Straussler
  • Disease, Gerstmann-Straussler
  • Diseases, Gerstmann-Straussler
  • Gerstmann Straussler Disease
  • Gerstmann Straussler Inherited Spongiform Encephalopathy
  • Gerstmann Straussler Scheinker Disease
  • Gerstmann Straussler Scheinker Syndrome
  • Gerstmann Straussler Syndrome
  • Gerstmann-Straussler Diseases
  • Inherited Spongiform Encephalopathy, Gerstmann Straussler

MeSH Record

Aspects Covered

36 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, congenital, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, transmission, urine, veterinary, virology.

MeSH Record

Indexing Annotation

a prion dis: do not confuse with GERSTMANN SYNDROME, a type of agnosia

MeSH Record

History Note

2000(1991); use SLOW VIRUS DISEASES 1988-1990; GERSTMANN-STRAUSSLER-SCHEINKER DISEASE was GERSTMANN-STRAUSSLER-SCHEINDER DISEASE see GERSTMANN-STRAUSSLER SYNDROME 1991

MeSH Record

Previous Indexing

  • Slow Virus Diseases (1988-1990)

MeSH Hierarchy

Tree Numbers

  1. C01w.207w.800w.350w
  2. C10w.228w.228w.800w.350w
  3. C10w.574w.500.425w
  4. C10w.574w.843w.400w
  5. C16w.320w.400.350w

AMA Style

References

  1. National Library of Medicine. Gerstmann-Straussler-Scheinker Disease. Medical Subject Headings (MeSH). 2026. Unique ID D016098. http://id.nlm.nih.gov/mesh/2026/D016098
  2. Gerstmann-Straussler-Scheinker Disease. In: Wikipedia. https://en.wikipedia.org/wiki/Gerstmann%E2%80%93Str%C3%A4ussler%E2%80%93Scheinker_syndrome
  3. Gerstmann-Straussler-Scheinker Disease. In: Wikidata. https://www.wikidata.org/wiki/Q383228