Prion Disorders and Sleep

Sleep disturbances are diagnostically relevant in the investigation of human prion diseases. Fatal familial insomnia is characterized by loss of slow-wave sleep, abnormal rapid eye movement (REM) states, and autonomic and motor hyperactivity without circadian rhythmicity. Abnormal REM sleep and deterioration of sleep organization also are found in sporadic Creutzfeldt-Jakob disease. Altered sleep also features in many prion diseases of animals, and prion diseases represent interesting models for sleep pathophysiology.