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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).

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.

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==References==