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J Clin Sleep Med. 2016 Jul 15;12(7):1033-9. doi: 10.5664/jcsm.5944.

Sleep Pathology in Creutzfeldt-Jakob Disease.

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Department of Neurology, Washington University School of Medicine, Saint Louis, MO.
Department of Neurology, University of Washington School of Medicine, Seattle, WA.
St. Luke's Hospital Sleep Medicine and Research Center, Saint Louis, MO.
Sleep Disorders Clinic, VA Portland Health Care System; Departments of Medicine, Neurology, and Behavioral Neuroscience, and the Oregon Institute of Occupational Health Sciences, Oregon Health & Science University, Portland, OR.



Associations between sleep and neurodegenerative diseases have become increasingly evident. This study aims to characterize the prevalence and type of sleep pathology in Creutzfeldt-Jakob disease (CJD), a rapidly progressive, fatal neurodegenerative disease.


In this observational cross-sectional cohort study, we performed a retrospective analysis of sleep signs and symptoms in a consecutive group of patients with definite CJD at a tertiary care medical center (n = 28). Polysomnography was performed in 14 patients.


Although only 5 of 28 patients carried a premorbid sleep diagnosis, signs/symptoms of sleep pathology were present in 25 patients. Eleven reported hypersomnia whereas 13 reported insomnia. Seven had restless legs symptoms and/or periodic limb movements of sleep, and nine reported parasomnias. Of the 14 patients who underwent polysomnography, 1 did not show sleep, 9 (69%) had poorly formed or absent sleep spindles and/or K-complexes, and 10 (77%) had sleep-disordered breathing. Of the 8 patients who experienced rapid eye movement (REM) sleep during the polysomnography, 3 (38%) showed REM sleep without atonia, and 2 patients met criteria for REM sleep behavior disorder. Median total sleep time was 226 (interquartile range [IQR] = 195-282) min. Median sleep efficiency was 58.5% (IQR = 41-65.5 %). Median REM time was 0.35% (IQR = 0-7.125%). Five patients (38%) demonstrated periodic limb movements during polysomnography. One case is presented.


Sleep pathology is common in CJD, and screening for sleep pathology is indicated in the evaluation of patients with rapidly progressive dementias. Early identification and treatment of sleep pathology may provide an intervenable target for CJD.


Creutzfeldt-Jakob disease; dementia; neurodegeneration; prion diseases; sleep disorders

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