威尔逊病的心血管和汗分泌自主神经功能障碍——与临床严重程度的相关性有限。

Cardiovascular and sudomotor autonomic dysfunction in Wilson's disease--limited correlation with clinical severity.

机构信息

Department of Neurology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India.

出版信息

Auton Neurosci. 2009 Dec 3;151(2):154-8. doi: 10.1016/j.autneu.2009.07.002. Epub 2009 Aug 8.

DOI:10.1016/j.autneu.2009.07.002

PMID:19665943

Abstract

BACKGROUND

Wilson's disease, a disorder of copper metabolism, results in abnormal accumulation of copper in liver, brain, kidney and cornea giving rise to protean manifestations. Wilson's disease predominantly affects the basal ganglia and brain stem nuclei which may cause autonomic dysfunction. Disturbances of autonomic nervous system have not received attention in Wilson's disease due to its rarity. The aim of this study was to evaluate autonomic cardiovascular reflexes in patients of Wilson's disease and to look for any relationship between autonomic nervous system disturbances and clinical severity of Wilson's disease.

MATERIALS AND METHODS

Cardiovascular autonomic reflexes were evaluated clinically and electrophysiologically in 30 patients of Wilson's disease with neurological onset and compared with equal number of age and gender matched healthy controls.

RESULTS

Significantly abnormal response to the Valsalva maneuver and RR interval variation was seen in patients compared to controls (p<0.05). Latency for sympathetic skin response was also significantly (p<0.02) prolonged in patient group. No specific correlation with clinical severity of Wilson's disease and autonomic dysfunction could be established.

CONCLUSION

Autonomic dysfunction occurs in Wilson's disease and affects parasympathetic more than the sympathetic functions.

摘要

背景

威尔逊病是一种铜代谢紊乱的疾病，导致铜在肝脏、大脑、肾脏和角膜中异常积聚，引起多种表现。威尔逊病主要影响基底神经节和脑干核，可能导致自主神经功能障碍。由于其罕见性，自主神经系统紊乱在威尔逊病中并未受到关注。本研究旨在评估威尔逊病患者的自主心血管反射，并寻找自主神经系统紊乱与威尔逊病临床严重程度之间的任何关系。