Gerstman syndrome

Gerstmann syndrome (GSS), which was first discovered and described by Gerstmann, Straussler, and Scheinker in 1936, is named after them. It is characterized by cerebellar ataxia accompanied by dementia and amyloid deposition in the brain, mostly familial. In 1981, Masters vaccination of animals confirmed the contagiousness of the disease. The average disease duration was 5 years. The average age of onset is 43-48 years (24-66 years), and it is a middle-aged progressive degenerative dementia of the cerebellum and spinal cord. In contrast to CJD, myoclonus is rare or absent. The diagnosis is confirmed by brain tissue examination and animal vaccination. The pathology shows that the amyloid plaques are the same as other TNDs. The nerve fibers are mainly in the cerebellum. Alzheimer's disease is similar to senile dementia. However, immunostaining with anti-Prpsc antibody can be positive. Negatives can be identified.