Variably protease-sensitive prionopathy

Variably protease-sensitive prionopathy
Protease sensitive and resistant prionopathies coexisting in this immunohistological test
SymptomsDementia, behavioral and psychiatric symptoms, ataxia, parkinsonism
Usual onset70 years
CausesPrion
Differential diagnosisAlzheimer's disease
TreatmentSupportive
PrognosisAverage survival 24 months
Frequency2-3 per 100 million people

Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) is a sporadic prion protein disease first described in an abstract for a conference on prions in 2006, and this study was published in a 2008 report on 11 cases. It was first identified as a distinct disease in 2010 by Zou W.Q. and coworkers from the United States National Prion Disease Pathology Surveillance Center. VPSPr shares similarities to other neurodegenerative disorders such as Creutzfeldt-Jakob disease and Alzheimer's, but there are unique markers of the disease in its clinical manifestations and electrophoretic profile.