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 Inherited Spongiform Encephalopathy; Gerstmann-Straussler-Scheinker Syndrome; Inherited Spongiform Encephalopathy, Gerstmann-Straussler; Gerstmann Straussler Inherited Spongiform Encephalopathy; Gerstmann Straussler Scheinker Disease; Gerstmann Straussler Scheinker Syndrome; Gerstmann Straussler Syndrome; Inherited Spongiform Encephalopathy, Gerstmann Straussler; Gerstmann-Straussler Syndrome
Networked: 26 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1. Creutzfeldt-Jakob Syndrome (Creutzfeldt-Jakob Disease)
2. Fatal Familial Insomnia
3. Prion Diseases (Transmissible Spongiform Encephalopathies)
4. Kuru
5. Neurodegenerative Diseases (Neurodegenerative Disease)


1. Kitamoto, T: 4 articles (03/2005 - 07/2000)
2. Jahandideh, Samad: 2 articles (01/2014 - 10/2011)
3. Zhi, Degui: 1 article (01/2014)
4. Srajan, Jain: 1 article (01/2013)
5. Rajasekaran, R: 1 article (01/2013)
6. Nagasundaram, N: 1 article (01/2013)
7. Rajith, B: 1 article (01/2013)
8. Debajyoti, C: 1 article (01/2013)
9. Doss, C George Priya: 1 article (01/2013)
10. Asadabadi, Ebrahim Barzegari: 1 article (10/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Gerstmann-Straussler-Scheinker Disease:
1. Amyloid (Amyloid Fibrils)IBA
2. PrionsIBA
3. Codon (Codons)IBA
4. alpha-SynucleinIBA
5. Heparan Sulfate Proteoglycans (Heparan Sulfate Proteoglycan)IBA
6. N-Acetylneuraminic Acid (Sialic Acid)IBA
7. AmberIBA
8. PrP 27-30 ProteinIBA
9. Complementary DNA (cDNA)IBA
10. Amyloid beta-Protein Precursor (Amyloid Protein Precursor)IBA

Therapies and Procedures

1. Transplants (Transplant)