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Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) is a sporadic prion protein disease identified in 2008〔(【引用サイトリンク】url=http://www.merckmanuals.com/professional/neurologic_disorders/prion_diseases/variably_protease-sensitive_prionopathy_vpspr.html )〕 and first described in 2010. VPSPr is very rare, occurring in 2 to 3/100 million people. (Nine cases had been identified in the UK by 2013.〔http://www.cjd.ed.ac.uk/documents/report22.pdf〕) 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 sensitve 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 yr, and duration of survival is 24 mo. About 40% of patients have a family history of dementia. Diagnosis is difficult. MRI, 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. ==See also== * Creutzfeld-Jakob disease 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Variably protease-sensitive prionopathy」の詳細全文を読む スポンサード リンク
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