The variable protease sensitive Prionopathie (VPSPr) is a rare prion disease (recognized in 2008).

VPSPr happens million people at 2-3 / 100.

The variable protease sensitive Prionopathie (VPSPr) is a rare prion disease (recognized in 2008). VPSPr happens million people at 2-3 / 100. VPSPr similar to the Gerstmann Scheinker disease (GSS) with respect to the properties of the abnormal prion protein (PrPSc). However, unlike GSS no mutations in the prion protein gene have been identified. The clinical manifestations differ from those of CJD, and that PrPSc is less resistant to cleavage by proteases; some varieties are more sensitive to proteases than others, hence the name: variable protease sensitive. Patients present with psychiatric symptoms, deficits in speech (aphasia and / or dysarthria) and cognitive impairment. It may develop ataxia and parkinsonism. The average age of onset is 70 years, and the survival time is 24 months. About 40% of patients have a family history of dementia. The diagnosis is difficult. MRI, EEG and tests to 14-3-3 protein and tau are usually not helpful, and there were no mutations in the coding region of the PrP gene observed. There is only supportive care for VPSPr.

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