Variably protease-sensitive prionopathy
| Variably protease-sensitive prionopathy | |
|---|---|
| Protease sensitive and resistant prionopathies coexisting in this immunohistological test | |
| Symptoms | Dementia, behavioral and psychiatric symptoms, ataxia, parkinsonism |
| Usual onset | 70 years |
| Causes | Prion |
| Differential diagnosis | Alzheimer's disease |
| Treatment | Supportive |
| Prognosis | Average survival 24 months |
| Frequency | 2-3 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.