运动神经元病患者自主神经功能与皮肤交感神经反应的观察

A study of autonomic dysfunction and sympathetic skin response in motor neuron disease

目的 调查运动神经元病（MND）患者的自主神经系统损害症状,并对患者进行皮肤交感反应（SSR）检查,对运动神经元病的自主神经受损情况进行评估.方法 收集来自2009年11月至2011年8月就诊于北京协和医院神经科门诊的MND病例.记录患者有无自主神经系统受损表现,包括：皮肤麻木或瘙痒;出汗异常包括出汗减少、冷汗或多汗;口干;流涎;皮温异常;体位性低血压;瞳孔大小或对光反射异常;便秘;排尿障碍;性功能障碍.对MND患者行SSR检查,测定结果参照我院肌电图室的正常标准,计算SSR异常率及电生理检查与临床表现之间的相关性.结果 共收集MND患者142例.患者中自主神经功能受损的出现率分别为：皮肤麻木76例（53.5％）;皮肤瘙痒22例（15.5％）;出汗异常15例（10.6％）;口干13例（9.1％）;流涎3例（2.1％）;皮温异常21例（14.8％）;体位性低血压3例（2.1％）;便秘23例（16.2％）;排尿障碍14例（9.9％）;性功能障碍2例（1.4％）.142例MND患者中,SSR正常91例（64.1％）,异常51例（35.9％）.上肢SSR异常12例（8.5％）,上肢潜伏期延长2例（1.4％）,波幅降低8例（5.6％）,波形消失4例（2.8％）;下肢SSR异常47例（33.1％）,下肢潜伏期延长4例（8.5％）,波幅降低31例（21.3％）,波形消失15例（10.6％）;上下肢均异常为7例（4.9％）,上下肢波形均消失3例（2.1％）.MND患者SSR与正常值比较,上肢潜伏期延长（P＜0.05）,上下肢波幅均显著降低（均P＜0.01）.ALS的各E1-Escorial诊断分型之间差异无统计学意义,下肢起病组异常率最高,差异有统计学意义.各自主神经功能损害表现与上、下肢SSR波幅、潜伏期之间无明显相关性.结论 MND可出现皮肤、腺体分泌、心血管系统、膀胱直肠括约肌等临床自主神经功能异常的表现.部分MND患者出现SSR的异常,表现为潜伏期延长和波幅降低.电生理改变与临床自主神经症状之间不具有相关性.下肢起病患者的SSR异常率较高.

Objective To explore the symptoms of autonomic dysfunction and sympathetic skin response （SSR） abnormality in patients of motor neuron disease （MND）.Methods The clinical features of autonomic dysfunction were collected as follows：dermal numbness or pruritus,parahidrosis,xerostomia,salivation,abnormal skin temperature,orthostatic hypotension,mydriasis,ptosis or abnormal pupillary light reflex,constipation,voiding dysfunction and sexual dysfunction.SSR was performed and the results were judged according to the normal range of our laboratory.Abnormality rate in MND patients was calculated.The relationship between clinical symptoms and SSR parameters were analyzed statistically.Results Among a total of 142 MND patients,the symptom incidences of autonomic dysfunction were as follows：dermal numbness （53.5%）,dermal pruritus （15.5%）,parahidrosis （10.6%）,xerostomia （9.1%）,salivation （2.1%）,abnormal skin temperature （14.8%）,orthostatic hypotension （2.1%）,constipation （16.2%）,voiding dysfunction （9.9%） and sexual dysfunction （1.4%）.Abnormal SSR was found in 51/142 （35.9%） patients,including 12 （8.5%） in palmar and 47 （33.1%） in plantar.The features of abnormal SSR included delayed latency of palmar （P 〈 0.05） and decreased amplitudes of both palmar and plantar compared with normal ranges （P 〈 0.01 respectively）.The group of patients with lumbosacral onset had a higher abnormal rate of SSR than those of other onset sites.There was no significant correlation between clinical symptoms and abnormal SSR parameters.Conclusion The patients of MND exhibit autonomic dysfunctions of skin,gland secretion,cardiovascular system and sphincters.Some of them show abnormal SSR with prolonged latency and decreased amplitude.The abnormalities of SSR are not related to clinical features of autonomic dysfunction.Those with an onset of lower limbs have a higher rate of abnormal SSR.