RT Book, Section A1 Honig, Lawrence S. A2 Brust, John C.M. SR Print(0) ID 1159972860 T1 Prion Diseases T2 CURRENT Diagnosis & Treatment: Neurology, 3e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259835315 LK neurology.mhmedical.com/content.aspx?aid=1159972860 RD 2024/10/14 AB Prion diseases are a group of less common neurodegenerative disorders characterized by rapidly progressive dementia. Few other disorders resemble the clinical syndrome (Table 29–1). Prion diseases result from accumulation in the brain of an abnormal conformation of the cellular protein called prion protein (PrP). Prion diseases are unusual among neurodegenerative disorders in that in addition to having sporadic and inherited forms, they also can rarely be transmissible through infection by iatrogenic or, in the case of variant CJD, by oral intake exposures.