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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
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In the early stages of Gerstmann-Straussler-Scheinker disease, patients may experience varying levels of ataxia (lack of muscle coordination), including clumsiness, unsteadiness, and difficulty walking. As the disease progresses, the ataxia becomes more pronounced and most patients develop dementia. Other symptoms may include dysarthria (slurring of speech), nystagmus (involuntary movements of the eyes), spasticity (rigid muscle tone), and visual disturbances, sometimes leading to blindness. Deafness also can occur. In some families, parkinsonian features are present.
Last updated: 7/22/2013
- 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.