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Harrison's Principles of Internal Medicine, 18e | Part 17. Neurologic Disorders > Section 2. Diseases of the Central Nervous System > | Chapter 383. Prion Diseases Sections: Prion Diseases: Introduction, Spectrum of Prion Diseases, Sporadic and Inherited Prion Diseases, Infectious Prion Diseases, Prion-Like Proteins Causing Otherneurodegenerative Diseases, Further Readings. Topics Discussed: prion diseases. Excerpt:"Prions are infectious proteins that cause degeneration of the central nervous system (CNS). Prion diseases are disorders of protein conformation, the most common of which in humans is called Creutzfeldt-Jakob disease (CJD). CJD typically presents with dementia and myoclonus, is relentlessly progressive, and generally causes death within a year 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...."
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