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Isabelle Arnulf, MD, PhD; Jørgen Nielsen, MD, PhD; Ebba Lohmann, MD; Johannes Schieffer, MD; Edward Wild, MD; Poul Jennum, MD, PhD; Eric Konofal, MD, PhD; Matthew Walker, FRCP, PhD; Delphine Oudiette, MS; Sarah Tabrizi, MRCP, PhD; Alexandra Durr, MD, PhD

Arch Neurol. 2008;65(4):482-488.

Background  Sleep disorders including insomnia, movements during sleep, and daytime sleepiness are common but poorly studied in Huntington disease (HD).

Objective  To evaluate the HD sleep-wake phenotype (including abnormal motor activity during sleep) in patients with various HD stages and the length of CAG repeats. Because a mild hypocretin deficiency has been found in the brains of some patients with HD (hereinafter referred to as HD patients), we also tested the HD patients for narcolepsy.

Design and Patients  Twenty-five HD patients (including 2 premanifest carriers) underwent clinical interview, nighttime video and sleep monitoring, and daytime multiple sleep latency tests. Their results were compared with those of patients with narcolepsy and control patients.

Results  The HD patients had frequent insomnia, earlier sleep onset, lower sleep efficiency, increased stage 1 sleep, delayed and shortened rapid eye movement (REM) sleep, and increased periodic leg movements. Three HD patients (12%) had REM sleep behavior disorders. No sleep abnormality correlated with CAG repeat length. Reduced REM sleep duration (but not REM sleep behavior disorders) was present in premanifest carriers and patients with very mild HD and worsened with disease severity. In contrast to narcoleptic patients, HD patients had no cataplexy, hypnagogic hallucinations, or sleep paralysis. Four HD patients had abnormally low (< 8 minutes) daytime sleep latencies, but none had multiple sleep-onset REM periods.

Conclusions  The sleep phenotype of HD includes insomnia, advanced sleep phase, periodic leg movements, REM sleep behavior disorders, and reduced REM sleep but not narcolepsy. Reduced REM sleep may precede chorea. Mutant huntingtin may exert an effect on REM sleep and motor control during sleep.

Author Affiliations: Pathologies du Sommeil, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), and U106, University Pierre and Marie Curie (Drs Arnulf and Konofal and Ms Oudiette), and Département de Génétique et Cytogénétique, Groupe Hospitalier Pitié-Salpêtrière, UMR S679, Neurologie et Thérapeutique Expérimentale (Drs Lohmann and Durr), Paris, France; Danish Center for Sleep Medicine (Dr Jennum) and Section of Neurogenetics (Dr Nielsen), Glostrup University Hospital, Glostrup, Denmark; Department of Neurology, University Hospital Rheinisch-Westfaelische Technische Hochschule Aachen, Aachen, Germany (Dr Schieffer); and Department of Neurodegenerative Diseases, Institute of Neurology, Queen Square, London, England (Drs Wild, Walker, and Tabrizi).

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