Valérie Cochen De Cock1,2, France Woimant3,4, Aurélia Poujois3,5. 1. Sleep and Neurology Unit, Beau Soleil Clinic, 119 avenue de Lodève, 34070, Montpellier, France. valerie.cochen@gmail.com. 2. EuroMov, Montpellier University, Montpellier, France. valerie.cochen@gmail.com. 3. French National Reference Centre for Wilson's Disease, Fondation Rothschild Hospital, Paris, France. 4. Neurology Department, Lariboisière University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. 5. Neurology Department, Fondation Rothschild Hospital, Paris, France.
Abstract
PURPOSE OF REVIEW: We aimed to review the sleep disorders described in Wilson's disease (WD), focusing on their mechanisms and treatments. RECENT FINDINGS: REM sleep behavior disorder or sleepiness can be warning signs of future WD. These early symptoms may significantly reduce the time to WD diagnosis. Early anti-copper therapies (chelators or zinc salts), reducing copper accumulation in the brain and though saving brain tissue, can allow the complete disappearance of these sleep disorders and of course improve the other symptoms of WD. Insomnia, restless legs syndrome (RLS), daytime sleepiness, cataplexy, and REM sleep behavior disorder (RBD) are present in WD and should be explored with video polysomnography and multiple sleep latency test. Suggested immobilization test could be useful in the diagnosis of RLS in WD. Motor and non-motor symptoms, dysautonomic dysfunctions, drugs, and lesions of the circuits regulating wake and sleep may be involved in the mechanisms of these sleep abnormalities. Adapted treatments should be proposed.
PURPOSE OF REVIEW: We aimed to review the sleep disorders described in Wilson's disease (WD), focusing on their mechanisms and treatments. RECENT FINDINGS:REM sleep behavior disorder or sleepiness can be warning signs of future WD. These early symptoms may significantly reduce the time to WD diagnosis. Early anti-copper therapies (chelators or zinc salts), reducing copper accumulation in the brain and though saving brain tissue, can allow the complete disappearance of these sleep disorders and of course improve the other symptoms of WD. Insomnia, restless legs syndrome (RLS), daytime sleepiness, cataplexy, and REM sleep behavior disorder (RBD) are present in WD and should be explored with video polysomnography and multiple sleep latency test. Suggested immobilization test could be useful in the diagnosis of RLS in WD. Motor and non-motor symptoms, dysautonomic dysfunctions, drugs, and lesions of the circuits regulating wake and sleep may be involved in the mechanisms of these sleep abnormalities. Adapted treatments should be proposed.
Entities:
Keywords:
Cataplexy; Insomnia; REM sleep behavior disorder; Restless legs syndrome; Sleepiness; Wilson’s disease
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