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)

Also Known As:

Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type; Gerstmann-Straussler Disease; Gerstmann-Straussler Inherited Spongiform Encephalopathy; 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; Gerstmann-Straussler Syndrome

Infections: 526564
- Central Nervous System Infections: 1390
  - Prion Diseases: 5345
    - Gerstmann-Straussler-Scheinker Disease: 51
Nervous System Diseases: 14178
- Neurodegenerative Diseases: 42472
  - Prion Diseases: 5345
    - Gerstmann-Straussler-Scheinker Disease: 51
  - Nervous System Heredodegenerative Disorders: 63
    - Gerstmann-Straussler-Scheinker Disease: 51
- Central Nervous System Diseases: 4213
  - Central Nervous System Infections: 1390
    - Prion Diseases: 5345
      - Gerstmann-Straussler-Scheinker Disease: 51
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Nervous System Heredodegenerative Disorders: 63
    - Gerstmann-Straussler-Scheinker Disease: 51

David L Vanik Selected Research

David L Vanik Research Topics

Disease

Drug/Important Bio-Agent (IBA)