brain, spinal cord, and nerve disorders
Gerstmann Sträussler Scheinker Disease (GSS)
Introduction
Gerstmann-Sträussler-Scheinker disease is a prion disease that causes loss of coordination followed by slow deterioration of mental function. The disease is fatal, usually in about 5 years. (See also Overview of Prion Diseases .) Like Creutzfeldt-Jakob Disease (CJD) , Gerstmann-Sträussler-Scheinker disease is a prion disease that may occur anywhere in the world. However, it is much less common than Creutzfeldt-Jakob disease. It usually begins earlier in life (affecting people in their 40s rather than in their 60s) and progresses more slowly (with an average life expectancy of 5 years rather than 6 months). Gerstmann-Sträussler-Scheinker disease is a Usually, the first symptoms are clumsiness and unsteadiness when walking. Muscle jerking ( Myoclonus ) is much less common than in Creutzfeldt-Jakob disease. Speaking becomes difficult (called dysarthria), and dementia develops. Nystagmus (rapid movement of the eyes in one direction, followed by a slower drift back to the original position) and deafness may develop. Muscle coordination is lost (called The diagnosis of Gerstmann-Sträussler-Scheinker disease is suggested by typical symptoms and a family history of the disease and is confirmed by genetic testing. No effective treatment is available. Treatment of Gerstmann-Sträussler-Scheinker disease focuses on relieving symptoms. 