PURPOSE OF REVIEW: Prion diseases are rare neurodegenerative diseases that are caused by abnormal pathogenic agents and can affect both humans and animals. These diseases are categorized as sporadic, inherited, or acquired by infection. Clinical manifestations include psychiatric symptoms, cognitive impairment, and parkinsonism, which are similar to those of other prion diseases and frontotemporal dementia variants. RECENT FINDINGS: More recently, scientists discovered a new sporadic prion disease called variably protease-sensitive prionopathy. SUMMARY: The following case discusses a patient presenting with sudden onset and rapid decline in cognitive, neurobehavioral, and motor functioning and his clinical journey including treatment interventions and diagnostic confirmation.
PURPOSE OF REVIEW: Prion diseases are rare neurodegenerative diseases that are caused by abnormal pathogenic agents and can affect both humans and animals. These diseases are categorized as sporadic, inherited, or acquired by infection. Clinical manifestations include psychiatric symptoms, cognitive impairment, and parkinsonism, which are similar to those of other prion diseases and frontotemporal dementia variants. RECENT FINDINGS: More recently, scientists discovered a new sporadic prion disease called variably protease-sensitive prionopathy. SUMMARY: The following case discusses a patient presenting with sudden onset and rapid decline in cognitive, neurobehavioral, and motor functioning and his clinical journey including treatment interventions and diagnostic confirmation.
Authors: Wen-Quan Zou; Gianfranco Puoti; Xiangzhu Xiao; Jue Yuan; Liuting Qing; Ignazio Cali; Miyuki Shimoji; Jan P M Langeveld; Rudy Castellani; Silvio Notari; Barbara Crain; Robert E Schmidt; Michael Geschwind; Stephen J Dearmond; Nigel J Cairns; Dennis Dickson; Lawrence Honig; Juan Maria Torres; James Mastrianni; Sabina Capellari; Giorgio Giaccone; Ermias D Belay; Lawrence B Schonberger; Mark Cohen; George Perry; Qingzhong Kong; Piero Parchi; Fabrizio Tagliavini; Pierluigi Gambetti Journal: Ann Neurol Date: 2010-08 Impact factor: 10.422
Authors: Pierluigi Gambetti; Zhiqian Dong; Jue Yuan; Xiangzhu Xiao; Mengjie Zheng; Amer Alshekhlee; Rudy Castellani; Mark Cohen; Marcelo A Barria; D Gonzalez-Romero; Ermias D Belay; Lawrence B Schonberger; Karen Marder; Carrie Harris; James R Burke; Thomas Montine; Thomas Wisniewski; Dennis W Dickson; Claudio Soto; Christine M Hulette; James A Mastrianni; Qingzhong Kong; Wen-Quan Zou Journal: Ann Neurol Date: 2008-06 Impact factor: 10.422
Authors: Matt Baker; Ian R Mackenzie; Stuart M Pickering-Brown; Jennifer Gass; Rosa Rademakers; Caroline Lindholm; Julie Snowden; Jennifer Adamson; A Dessa Sadovnick; Sara Rollinson; Ashley Cannon; Emily Dwosh; David Neary; Stacey Melquist; Anna Richardson; Dennis Dickson; Zdenek Berger; Jason Eriksen; Todd Robinson; Cynthia Zehr; Chad A Dickey; Richard Crook; Eileen McGowan; David Mann; Bradley Boeve; Howard Feldman; Mike Hutton Journal: Nature Date: 2006-07-16 Impact factor: 49.962
Authors: Abigail B Diack; Diane L Ritchie; Alexander H Peden; Deborah Brown; Aileen Boyle; Laura Morabito; David Maclennan; Paul Burgoyne; Casper Jansen; Richard S Knight; Pedro Piccardo; James W Ironside; Jean C Manson Journal: Emerg Infect Dis Date: 2014-12 Impact factor: 6.883