TY - CHAP M1 - Book, Section TI - PRION DISEASES A1 - Prusiner, Stanley B. A1 - Miller, Bruce L. A2 - Hauser, Stephen L. A2 - Josephson, S. Andrew PY - 2018 T2 - Harrison's Neurology in Clinical Medicine, 4e AB - Prions are proteins that adopt an alternative conformation, which becomes self-propagating. Some prions cause degeneration of the central nervous system (CNS). Once relegated to causing a group of rare disorders of the CNS such as Creutzfeldt-Jakob disease (CJD), prions—as mounting evidence shows—also appear to play a key role in more common illnesses such as Alzheimer’s disease (AD) and Parkinson’s disease (PD). While CJD is caused by the accumulation of PrPSc, increasing data argue that Aβ prions cause AD, α-synuclein prions cause PD, and tau prions cause the frontotemporal dementias (FTDs). In this chapter, we confine our discussion to CJD, which typically presents with a rapidly progressive dementia as well as motor abnormalities. The illness is relentlessly progressive and generally causes death within 9 months of onset. Most CJD patients are between 50 and 75 years of age; however, patients as young as 17 and as old as 83 have been recorded. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/11/12 UR - neurology.mhmedical.com/content.aspx?aid=1145769904 ER -