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Nervous System Diseases > Neurodegenerative Diseases > Prion Diseases > Gerstmann-Straussler-Scheinker Disease
Nervous System Diseases > Central Nervous System Diseases > Central Nervous System Infections > Prion Diseases > Gerstmann-Straussler-Scheinker Disease
Infections > Central Nervous System Infections > Prion Diseases > Gerstmann-Straussler-Scheinker Disease
Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Heredodegenerative Disorders, Nervous System > Gerstmann-Straussler-Scheinker Disease
Nervous System Diseases > Neurodegenerative Diseases > Heredodegenerative Disorders, Nervous System > Gerstmann-Straussler-Scheinker Disease

Preferred term

Gerstmann-Straussler-Scheinker Disease  

Type

  • mesh:Descriptor

Definition

  • 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)

Broader concept

Entry terms

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

Allowable Qualifier(s)

In other languages

  • Syndrome de Gerstmann-Sträussler-Scheinker

    French

  • Encéphalopathie spongiforme subaiguë type Gerstmann-Straussler
  • Encéphalopathie spongiforme subaigüe type Gerstmann-Straussler
  • Syndrome de Gerstmann-Sträussler

URI

http://data.loterre.fr/ark:/67375/JVR-M5FJ2B39-T

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