Abstract
Huntington’s chorea (Huntington’s disease, HD) is a genetic disorder caused by autosomal dominant mutation, leading to progressive neurodegenerative changes in the central nervous system. Involuntary movements such as chorea occur typically in HD patients, accompanied by progressive cognitive and psychiatric disturbances. Other common symptoms of HD are circadian and sleep abnormalities, which are observed from the earliest stages of the disease or even before the occurrence of clinical symptoms. The most common sleep problems reported by HD patients include insomnia, difficulties in falling asleep, frequent nocturnal awakenings, and excessive daytime sleepiness. Also, specific changes in sleep architecture have been identified in HD. In this paper, we review studies on sleep and circadian rhythm disorders in HD. We outline findings concerning sleep patterns and disturbances of circadian rhythms in HD patients, as well as the role of psychiatric disorders and motor disorders in HD patients’ sleep problems. We also discuss problems related to the different methods of diagnosing sleep disorders in HD. Furthermore, the adverse effects of medication used for the treatment of core HD symptoms as one of the sources of sleep disturbances in HD are emphasized. In conclusion, the diversity and complexity of the determinants of sleep and circadian rhythm disorders in HD are highlighted. Finally, the relevance of effective treatment to improve patients’ functioning and quality of life as well as the potential relief of their cognitive and emotional symptoms is addressed.
Highlights
Huntington’s chorea (Huntington’s disease, HD) is a genetic disorder caused by autosomal dominant mutation, leading to progressive neurodegenerative changes in the central nervous system
We review the literature on sleep disorders and circadian rhythm disturbances in HD patients
Different patterns of sleep disturbances are observed in HD patients: insomnia, difficulties in falling asleep, frequent nocturnal awakenings, and excessive daytime sleepiness are the most common sleep problems reported by patients with HD
Summary
Huntington’s chorea (Huntington’s disease, HD) is a genetic disorder caused by autosomal dominant mutation, leading to progressive neurodegenerative changes in the central nervous system. The age of onset of HD symptoms strongly correlates with the number of CAG trinucleotide repeats in HTT. Sleep disorders in HD in the mutation carrier causes earlier onset of the disease, more severe cognitive impairments, severe progression of degenerative changes, and worse prognosis [9]. The most common cognitive deficits in HD include attention [15, 16] and executive function disorders manifested by difficulties in taking decisions, planning and execution of complex activities, reduced flexibility of thinking and behavior, as well as a tendency to perseveration [17, 18]. The number of CAG repeats in most studies did not correlate with the occurrence of sleep disorders [27]; they were partially correlated with the duration of the disease and the severity of clinical symptoms [28]. We performed a search of online electronic databases (PubMed, MEDLINE, Scopus, and Google Scholar) that was most recently updated on October 10, 2018, followed by analysis of reference lists for additional articles
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