Gerstmann-Straussler-Scheinker disease (GSS) is a rare condition and neurodegenerative disorder of the brain. In almost all cases it is being inherited and can be found only in few families around the world. The symptoms of the disease usually start occurring between the age of 35 and 55. Suring early stages the patients experience varied levels of lack of muscle coordination or ataxia, including clumsiness, unsteadiness and problem in walking. As the diseases progresses, ataxia becomes more significant and most patient suffer from dementia. Other symptoms of the disease include dysartria, nystagmu, spasticity and visual disturbances at times which can lead to blindness. Some patients may also suffer from deafness and in some cases parkinsons features are also present.
GSS is a slowly progressing disease which usually lasts from 2-10 years. This disease can cause severe disabilities and eventual death. The patient goes into a coma or has a secondary infection such as aspiration pneumonia due to an impaired ability to swallow.
There is no cure for GSS. There is no known treatment for slow progression of the diseases also. The recent therapies are can relieve the symptoms and can make the patient comfortable.
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