Variably protease-sensitive prionopathy
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 is a transmissible spongiform encephalopathy in humans. Diagnosis is difficult, as pathognomonic signs on MRI such as cortical ribboning or hockey stick sign, periodic sharp wave complexes on EEG, and tests for 14-3-3 protein and tau protein are usually not helpful, and no mutations have been observed in the coding region of the PrP gene, unlike CJD and Variant CJD. Symptoms are similar to Creutzfeldt-Jakob Disease. Behavior and mood changes, expressed as disinhibition, euphoria, and impulsivity or loss of interest and apathy, were the most frequent (80% of the cases). The disease is always fatal.
