Other Names for this Disease
- Amyloidosis cerebral with spongiform encephalopathy
- Cerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous system
- Encephalopathy subacute spongiform Gerstmann-Straussler type
- Gerstmann Straussler Scheinker syndrome
Your QuestionI may be at-risk to develop Gerstmann-Straussler-Scheinker disease. Is there a test that can be performed to provide me with a definitive diagnosis, even if I don't currently have symptoms?
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Questions on this page
- What is Gerstmann-Straussler-Scheinker disease?
- What are the symptoms of Gerstmann-Straussler-Scheinker disease?
- How is Gerstmann-Straussler-Scheinker disease inherited?
- What genes are associated with Gerstmann-Straussler-Scheinker disease?
- How is Gerstmann-Straussler-Scheinker disease diagnosed?
- How might Gerstmann-Straussler-Scheinker disease be treated?
- What is the prognosis for individuals with Gerstmann-Straussler-Scheinker disease?
Testing of at-risk asymptomatic adults for Gerstmann-Straussler-Scheinker disease is done through genetic testing which may identify the presence of the disease-causing mutation in the PRNP gene. This testing is not useful in predicting age of onset, severity, type of symptoms, or rate of progression in asymptomatic individuals. Testing for the disease-causing mutation in the absence of definite symptoms of the disease is called predictive testing.
Evaluation and testing for Gerstmann-Straussler-Scheinker disease can be coordinated by a genetics professional at a genetics clinic. Visit the Resources section to find a list of online resources that can assist you in finding a genetics clinic near you.
- NINDS Gerstmann-Straussler-Scheinker Disease Information Page. National Institute of Neurological Disorders and Stroke (NINDS). February 13, 2007; http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008.
- Prion disease. Genetics Home Reference (GHR). May 2007; http://ghr.nlm.nih.gov/condition=priondisease. Accessed 3/21/2008.
- Mastrianni JA. Genetic Prion Diseases. GeneReviews. September 7, 2010; http://www.ncbi.nlm.nih.gov/books/NBK1229/. Accessed 7/22/2013.