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Concept information

Preferred term

Gerstmann-Straussler-Scheinker Disease  

Type

  • Topical Descriptor

Entry terms

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

Note

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

Scope note

  • 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)

History note

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

In other languages

URI

http://www.yso.fi/onto/mesh/D016098

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