You Zhifei, Xu Hui, Wu Zhonghua, You Zhengchen
Department of Neurology, Taizhou People's Hospital, Taizhou, Jiangsu, China.
Department of Neurosurgery, The Sixth People's Hospital of Nantong, Nantong, Jiangsu, China.
Neurol Sci. 2023 Jan;44(1):209-214. doi: 10.1007/s10072-022-06401-4. Epub 2022 Sep 15.
To examine the clinical characteristics and influencing factors related to sleep disorders in patients with Wilson's disease (WD), and investigate its potential mechanisms.
A total of 150 patients with WD (76 hepatic, 42 neurological, 32 asymptomatic form) and 150 age- and sex-matched control subjects were investigated using 3 standardized sleep questionnaires. Differences among 3 subtypes were discussed.
The mean Parkinson's disease sleep scale (PDSS) score of WD was lower than the controls (Z = - 4.426, P = 0.000), and their mean Epworth Sleepiness Scale (ESS) score as well as Pittsburgh sleep quality index (PSQI) score of WD was higher than that of the controls (t = 2.005, P = 0.048; t = 3.342, P = 0.001). The incidence of excessive daytime sleepiness (EDS) in WD group were significantly higher than the controls (X = 6.064, P = 0.014). Further analysis showed that total PDSS score of neurologic presentation group was lower than others (X = 6.131, P = 0.047), while the ESS score was higher (F = 3.817, P = 0.029). UWDRS showed a negative correlation with PDSS (r = - 0.440, P = 0.022) and has a higher negative correlation with PDSS in neurologic presentation group (r = - 0.732, P = 0.000).
Patients with WD often suffer from sleep disturbances, mainly characterized by difficulty falling asleep, difficulty staying asleep, nocturnal motor symptoms (numbness, cramps, tremor), and daytime dozing. And the incidence of EDS is significantly higher than that of the controls. Sleep quality is worse in patients with WD of neurologic presentation than the other two groups. Furthermore, the worse of the symptoms, patients with WD suffer more serious of the sleep disorders especially in neurologic presentation group.
探讨肝豆状核变性（WD）患者睡眠障碍的临床特征及相关影响因素，并研究其潜在机制。
使用3份标准化睡眠问卷对150例WD患者（76例肝脏型、42例神经型、32例无症状型）和150例年龄及性别匹配的对照者进行调查。讨论3种亚型之间的差异。
WD患者的帕金森病睡眠量表（PDSS）平均得分低于对照组（Z = -4.426，P = 0.000），其爱泼沃斯思睡量表（ESS）平均得分以及匹兹堡睡眠质量指数（PSQI）得分高于对照组（t = 2.005，P = 0.048；t = 3.342，P = 0.001）。WD组日间过度嗜睡（EDS）的发生率显著高于对照组（X = 6.064，P = 0.014）。进一步分析显示，神经型表现组的PDSS总得分低于其他组（X = 6.131，P = 0.047），而ESS得分更高（F = 3.817，P = 0.029）。统一帕金森病评定量表（UWDRS）与PDSS呈负相关（r = -0.440，P = 0.022），在神经型表现组中与PDSS的负相关性更高（r = -0.732，P = 0.000）。
WD患者常伴有睡眠障碍，主要表现为入睡困难、睡眠维持困难、夜间运动症状（麻木、抽筋、震颤）及日间打瞌睡。且EDS的发生率显著高于对照组。神经型表现的WD患者的睡眠质量比其他两组更差。此外，症状越严重，WD患者的睡眠障碍越严重，尤其是在神经型表现组。
