Variably protease-sensitive prionopathy
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|Variably protease-sensitive prionopathy|
|Protease senstitive and resistant prionopathies coexisting in this immunohistological test|
Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) is a sporadic prion protein disease identified in 2008 and first described in 2010 by Zou W.Q. and coworkers from the United States National Prion Disease Pathology Surveillance Center.
VPSPr is very rare, occurring in just 2 or 3 out of every 100 million people. As of 2018, fourteen cases have been reported in the UK. It has similarities to Creutzfeldt–Jakob disease, but clinical manifestations differ somewhat, and the abnormal prion protein (PrP) is less resistant to digestion by proteases; some variants are more sensitive to proteases than others, hence the name: variably protease-sensitive.
Patients present with psychiatric symptoms, speech deficits (aphasia and/or dysarthria) and cognitive impairment. Ataxia and parkinsonism can develop. Average age at onset is 70 years, and duration of survival is 24 months. About 40% of patients have a family history of dementia.
- "The Merck Manual - Variably Protease-Sensitive Prionopathy (VPSPr)". Merck Sharp & Dohme Corp., a subsidiary of Merck & Co. Retrieved 16 November 2014.
- Zou, W.; Puoti, G.; Xiao, X.; Yuan, J.; Qing, L.; Cali, I.; Shimoji, M.; Langeveld, J.; Castellani, R.; Notari, S.; Crain, B.; Schmidt, R. E.; Geschwind, M.; Dearmond, S. J.; Cairns, N. J.; Dickson, D.; Honig, L.; Torres, J. M.; Mastrianni, J.; Capellari, S.; Giaccone, G.; Belay, E. D.; Schonberger, L. B.; Cohen, M.; Perry, G.; Kong, Q.; Parchi, P.; Tagliavini, F.; Gambetti, P. (2010). "Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein". Annals of Neurology. 68 (2): 162–172. doi:10.1002/ana.22094. PMC 3032610. PMID 20695009.
- "Variably Protease-Sensitive Prionopathy (VPSPr) - Neurologic Disorders - Merck Manuals Professional Edition". Merck Manuals Professional Edition. Retrieved 2018-03-04.
- "Creutzfeldt - Jakob disease in the UK (By calendar year)" (PDF). Retrieved 4 July 2018.
- Caroline Parkinson (13 August 2010). "Brain disease could affect more people, research finds". BBC News.
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