Sleep disorders in hepatolenticular degeneration: a primary study

<p><strong>Objective </strong> To investigate the characteristics and potential mechanisms of sleep disorders in patients with hepatolenticular degeneration (HLD), also known as Wilson's disease (WD). <strong>Methods</strong> Twenty-six patients with HLD completed a questionnaire concerning Parkinson's Disease Sleep Scale (PDSS), Epworth Sleepiness Scale (ESS) and Pittsburgh Sleep Quality Index (PSQI). <strong>Results</strong> The sleep quality of patients with HLD was significantly worse than controls. Their overall PDSS score was lower than the controls (<em>P</em> = 0.000), including sub tests such as worse overall quality of sleep at night (<em>P</em> = 0.010), more difficulty falling asleep (<em>P</em> = 0.009), increased difficulty staying asleep (<em>P</em> = 0.013), increased tremor (<em>P</em> = 0.005), more tiredness and sleepiness after waking up in the morning (<em>P</em> = 0.042) and excessive daytime sleepiness (EDS, <em>P</em> = 0.028). The mean ESS (<em>P</em> = 0.009) as well as PSQI (<em>P</em> = 0.005) scores of HLD patients was higher than those of the controls. Further analysis showed that total PDSS score of neurological HLD group was lower than hepatic HLD group and asymptomatic HLD group (<em>P</em> = 0.046), including sub tests such as worse overall quality of sleep at night (<em>P</em> = 0.021), increased difficulty falling asleep (<em>P</em> = 0.009) and more difficulty staying asleep (<em>P</em> = 0.002). <strong>Conclusions</strong> Patients with HLD often suffer from sleep disturbances, mainly including difficulty falling asleep, difficulty staying asleep at night and EDS. Besides, sleep quality was significantly worse in patients with neurological HLD than that of hepatic HLD and asymptomatic HLD.</p><p> </p><p><strong>DOI: </strong>10.3969/j.issn.1672-6731.2016.05.007