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Gerstmann-Straussler-Scheinker Disease Reviewed 03-26-2003 Get Web page suited for printing Email this to a friend or colleague Studies with patients Table of Contents (click to jump to sections) What are Gerstmann-Straussler-Scheinker Disease?Is there any treatment? What is the prognosis? What research is being done? Organizations What are Gerstmann-Straussler-Scheinker Disease? Gerstmann-Straussler-Scheinker disease (GSS) is an extremely rare, neurodegenerative brain disorder. It is almost always inherited and is found in only a few families around the world. Onset of the disease usually occurs between the ages of 35 and 55. In the early stages, 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. GSS belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Other TSEs include Creutzfeldt-Jakob disease, kuru, and fatal familial insomnia. Is there any treatment? What is the prognosis? What research is being done? National Organization for Rare Disorders (NORD)
National Institute on Aging (NIA)
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