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Gerstmann syndrome

Gerstmann syndrome (GSS) was first discovered and described by Gerstmann, straussler and scheinker in 1936, so it was named after them. It is characterized by cerebellar ataxia with dementia and amyloid deposition in the brain, mostly familial. In 1981, masters inoculated animals confirmed the infectivity of the disease. The average course of disease was 5 years. The average age of onset was 43 ~ 48 years (24 ~ 66 years). It was a middle-aged progressive cerebellar spinal cord degenerative dementia. In contrast to CJD, myoclonus is rare or absent. The diagnosis depends on brain tissue examination and animal vaccination. Pathology showed that outside the amyloid plaque, like other TNDs, nerve fibers were mainly surrounded in the cerebellum, which was similar to Alzheimer's disease, but it could be positive by anti PrPSc antibody immunostaining β- Amyloid antibody staining was negative for identification.


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