Sleep Disorders in Human Prionopathies

Purpose of Review: Sleep disorders are common in human prionopathies, such as sporadic Creutzfeldt-Jakob disease and fatal familial insomnia, and often manifest early in the disease course. Recent Findings: Clinical manifestations of sleep pathology in these disorders are variable and include insomnia, sleep disordered breathing, rapid eye movement sleep behavior disorder, and hypersomnia, all of which likely result from the rapid neurodegeneration of anatomic structures critical to sleep physiology. Summary: Given their high prevalence in human prionopathies, sleep disorders/disturbances may be of diagnostic utility and/or serve as potential biomarkers of human prion disease, and a thorough screen for sleep disturbances should be performed by all clinicians evaluating patients with rapidly progressive dementias. While limited data exists on the subject, treatment of sleep disturbances in patients with prion disease may improve quality of life, for both patients and caretakers.