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先兆期和早期亨廷顿病的昼夜节律和自主神经功能障碍。

Circadian rhythm and autonomic dysfunction in presymptomatic and early Huntington's disease.

机构信息

Neurology Department, Hospital Clínico Universitario Lozano Blesa, San Juan Bosco 15, 50009 Zaragoza, Spain; Research Group of Movement Disorders and Headache (GIIS070), Aragon Institute of Health Sciences, San Juan Bosco 13, 50009 Zaragoza, Spain.

Neurology Department, Movement Disorders Unit, Hospital de la Santa Creu i Sant Pau, Carrer de Sant Quintí 89, 08026 Barcelona, Spain; Biomedical Research Institute Sant Pau (IIB-Sant Pau), Carrer de Sant Quintí 89, Barcelona, Spain; Centro de Investigación Biomedica en Red-Enfermedades Neurodegenerativas (CIBERNED), Spain; Instituto de Salud Carlos III, Spain.

出版信息

Parkinsonism Relat Disord. 2017 Nov;44:95-100. doi: 10.1016/j.parkreldis.2017.09.013. Epub 2017 Sep 15.

DOI:10.1016/j.parkreldis.2017.09.013
PMID:28935191
Abstract

INTRODUCTION

Sleep and circadian rhythm disturbances are common in patients with neurodegenerative diseases such as Huntington's disease (HD). The aim of this study was to evaluate variability in circadian blood pressure (BP) to determine the association between abnormal circadian BP and sleep quality in patients with HD.

METHODS

Cross-sectional, multicenter study of 38 HD mutation carriers (23 premanifest and 15 early stage patients) who were compared to 38 age- and sex-matched controls. BP was evaluated by ambulatory blood pressure monitoring (ABPM). Based on the percentage decrease in nocturnal BP, subjects were classified as either dippers (≥10%) or non-dippers (<10%). Sleep quality and daytime sleepiness were measured, respectively, using the Pittsburgh Sleep Quality Index (PSQI) and the Epworth Daytime Sleepiness Scale (ESS) and the scores on these indices were correlated with the ABPM findings.

RESULTS

Sixty-three percent HD mutation carriers were non-dippers (86.7% of the symptomatic and 47.8% of the premanifest patients) versus 23.7% of controls (p = 0.001). In the HD group, sleep quality was significantly more impaired (PSQI>5) (p = 0.016) with more excessive daytime sleepiness (ESS>9) (p = 0.001) than in the control group. Nocturnal non-dipping was associated with worse sleep quality in patients (p = 0.011) but not in controls.

CONCLUSION

These results show that patients with HD present early disturbances in the circadian rhythm of BP and that this altered nocturnal BP is associated with poor sleep quality. These findings suggest the potential role of subtle hypothalamic dysfunction in this population.

摘要

简介

睡眠和昼夜节律紊乱在神经退行性疾病患者中很常见,如亨廷顿病(HD)。本研究旨在评估昼夜血压(BP)的变异性,以确定 HD 患者异常昼夜 BP 与睡眠质量之间的关系。

方法

这是一项横断面、多中心研究,共纳入 38 名 HD 基因突变携带者(23 名前显症和 15 名早期患者),并与 38 名年龄和性别匹配的对照者进行比较。通过动态血压监测(ABPM)评估血压。根据夜间 BP 下降的百分比,将受试者分为杓型(≥10%)或非杓型(<10%)。分别采用匹兹堡睡眠质量指数(PSQI)和 Epworth 白天嗜睡量表(ESS)评估睡眠质量和白天嗜睡程度,并用这些指数的评分与 ABPM 结果进行相关性分析。

结果

63%的 HD 基因突变携带者为非杓型(症状性患者中为 86.7%,前显症患者中为 47.8%),而对照组为 23.7%(p=0.001)。与对照组相比,HD 组患者的睡眠质量明显更差(PSQI>5)(p=0.016),白天嗜睡程度更严重(ESS>9)(p=0.001)。夜间非杓型与患者的睡眠质量较差相关(p=0.011),但与对照组无关。

结论

这些结果表明,HD 患者的血压昼夜节律早期出现紊乱,这种改变的夜间血压与睡眠质量差有关。这些发现提示在该人群中下丘脑功能轻微障碍的潜在作用。

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