TY - CHAP M1 - Book, Section TI - Prion Diseases A1 - Honig, Lawrence S. A2 - Brust, John C.M. PY - 2019 T2 - CURRENT Diagnosis & Treatment: Neurology, 3e 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/10 UR - neurology.mhmedical.com/content.aspx?aid=1159972860 ER -