Dulce Neutel1, Maya Tchikviladzé2, Perrine Charles3, Smaranda Leu-Semenescu4, Emmanuel Roze5, Alexandra Durr6, Isabelle Arnulf7. 1. APHP, Sleep Disorder Unit, Pitié-Salpêtrière University Hospital, Paris, France. 2. APHP, Département de Génétique, Pitié-Salpêtrière University Hospital, Paris, France. 3. Reference Center for Huntington disease, Pitié-Salpêtrière University Hospital, Paris, France. 4. APHP, Sleep Disorder Unit, Pitié-Salpêtrière University Hospital, Paris, France; Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, INSERM UMR_S 975, CNRS UMR 7225, Université Pierre et Marie Curie, Paris 6, Paris, France. 5. Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, INSERM UMR_S 975, CNRS UMR 7225, Université Pierre et Marie Curie, Paris 6, Paris, France. 6. APHP, Département de Génétique, Pitié-Salpêtrière University Hospital, Paris, France; Reference Center for Huntington disease, Pitié-Salpêtrière University Hospital, Paris, France; Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, INSERM UMR_S 975, CNRS UMR 7225, Université Pierre et Marie Curie, Paris 6, Paris, France. 7. APHP, Sleep Disorder Unit, Pitié-Salpêtrière University Hospital, Paris, France; Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, INSERM UMR_S 975, CNRS UMR 7225, Université Pierre et Marie Curie, Paris 6, Paris, France. Electronic address: isabelle.arnulf@psl.aphp.fr.
Abstract
BACKGROUND: Patients with Huntington disease (HD) and their spouses often complain of agitation during sleep, but the causes are mostly unknown. OBJECTIVE: To evaluate sleep and nocturnal movements in patients with various HD stages and CAG repeats length. METHODS: The clinical features and sleep studies of 29 patients with HD were retrospectively collected (11 referred for genotype-phenotype correlations and 18 for agitation during sleep) and compared with those of 29 age- and sex-matched healthy controls. All patients had videopolysomnography, but the movements during arousals were re-analyzed in six patients with HD with stored video. RESULTS: The patients had a longer total sleep period and REM sleep onset latency, but no other differences in sleep than controls. There was no correlation between CAG repeat length and sleep measures, but total sleep time and sleep efficiency were lower in the subgroup with moderate than milder form of HD. Periodic limb movements and REM sleep behavior disorders were excluded, although 2/29 patients had abnormal REM sleep without atonia. In contrast, they had clumsy and opisthotonos-like movements during arousals from non-REM or REM sleep. Some movements were violent and harmful. They might consist of voluntary movements inappropriately involving the proximal part of the limbs on a background of exaggerated hypotonia. Giant (>65 mcV) sleep spindles were observed in seven (24%) patients with HD and one control. CONCLUSION: The nocturnal agitation in patients with HD seems related to anosognostic voluntary movements on arousals, rather than to REM sleep behavior disorder and other sleep problems.
BACKGROUND:Patients with Huntington disease (HD) and their spouses often complain of agitation during sleep, but the causes are mostly unknown. OBJECTIVE: To evaluate sleep and nocturnal movements in patients with various HD stages and CAG repeats length. METHODS: The clinical features and sleep studies of 29 patients with HD were retrospectively collected (11 referred for genotype-phenotype correlations and 18 for agitation during sleep) and compared with those of 29 age- and sex-matched healthy controls. All patients had videopolysomnography, but the movements during arousals were re-analyzed in six patients with HD with stored video. RESULTS: The patients had a longer total sleep period and REM sleep onset latency, but no other differences in sleep than controls. There was no correlation between CAG repeat length and sleep measures, but total sleep time and sleep efficiency were lower in the subgroup with moderate than milder form of HD. Periodic limb movements and REM sleep behavior disorders were excluded, although 2/29 patients had abnormal REM sleep without atonia. In contrast, they had clumsy and opisthotonos-like movements during arousals from non-REM or REM sleep. Some movements were violent and harmful. They might consist of voluntary movements inappropriately involving the proximal part of the limbs on a background of exaggerated hypotonia. Giant (>65 mcV) sleep spindles were observed in seven (24%) patients with HD and one control. CONCLUSION: The nocturnal agitation in patients with HD seems related to anosognostic voluntary movements on arousals, rather than to REM sleep behavior disorder and other sleep problems.
Authors: Ye Zhang; Rong Ren; Linghui Yang; Junying Zhou; Yun Li; Jie Shi; Lin Lu; Larry D Sanford; Xiangdong Tang Journal: Sleep Date: 2019-10-09 Impact factor: 5.849
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