Prion Disorders: Creutzfeldt-Jakob Disease and Related Disorders
- DOI:
- 10.1093/med/9780190244927.003.0079
Prion disorders, also known as transmissible spongiform encephalopathies (TSEs), are a group of universally fatal human and animal diseases that cause rapid degeneration of brain neurons (Table 69.1). The essential pathogenesis is called the prion theory: a conformational change of host-derived prion protein (PrP), that propagates further conformational change, which amplifies the process that selectively affects neurons. TSEs may be sporadic, inherited, or spread in an acquired manner like an infectious agent. The acquired manner is least frequent. This chapter reviews the molecular theory of prions and the pathology, clinical presentation, and management of various prion diseases.
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