中华医学杂志
中華醫學雜誌
중화의학잡지
National Medical Journal of China
2014年
41期
3224-3228
,共5页
王心宁%崔丽英%刘明生%管宇宙%李本红%杜华
王心寧%崔麗英%劉明生%管宇宙%李本紅%杜華
왕심저%최려영%류명생%관우주%리본홍%두화
运动神经元病%自主神经系统疾病%交感神经系统
運動神經元病%自主神經繫統疾病%交感神經繫統
운동신경원병%자주신경계통질병%교감신경계통
Motor neuron disease%Autonomic nervous system diseases%Sympathetic nervous system
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.
目的 調查運動神經元病(MND)患者的自主神經繫統損害癥狀,併對患者進行皮膚交感反應(SSR)檢查,對運動神經元病的自主神經受損情況進行評估.方法 收集來自2009年11月至2011年8月就診于北京協和醫院神經科門診的MND病例.記錄患者有無自主神經繫統受損錶現,包括:皮膚痳木或瘙癢;齣汗異常包括齣汗減少、冷汗或多汗;口榦;流涎;皮溫異常;體位性低血壓;瞳孔大小或對光反射異常;便祕;排尿障礙;性功能障礙.對MND患者行SSR檢查,測定結果參照我院肌電圖室的正常標準,計算SSR異常率及電生理檢查與臨床錶現之間的相關性.結果 共收集MND患者142例.患者中自主神經功能受損的齣現率分彆為:皮膚痳木76例(53.5％);皮膚瘙癢22例(15.5％);齣汗異常15例(10.6％);口榦13例(9.1％);流涎3例(2.1％);皮溫異常21例(14.8％);體位性低血壓3例(2.1％);便祕23例(16.2％);排尿障礙14例(9.9％);性功能障礙2例(1.4％).142例MND患者中,SSR正常91例(64.1％),異常51例(35.9％).上肢SSR異常12例(8.5％),上肢潛伏期延長2例(1.4％),波幅降低8例(5.6％),波形消失4例(2.8％);下肢SSR異常47例(33.1％),下肢潛伏期延長4例(8.5％),波幅降低31例(21.3％),波形消失15例(10.6％);上下肢均異常為7例(4.9％),上下肢波形均消失3例(2.1％).MND患者SSR與正常值比較,上肢潛伏期延長(P＜0.05),上下肢波幅均顯著降低(均P＜0.01).ALS的各E1-Escorial診斷分型之間差異無統計學意義,下肢起病組異常率最高,差異有統計學意義.各自主神經功能損害錶現與上、下肢SSR波幅、潛伏期之間無明顯相關性.結論 MND可齣現皮膚、腺體分泌、心血管繫統、膀胱直腸括約肌等臨床自主神經功能異常的錶現.部分MND患者齣現SSR的異常,錶現為潛伏期延長和波幅降低.電生理改變與臨床自主神經癥狀之間不具有相關性.下肢起病患者的SSR異常率較高.
목적 조사운동신경원병(MND)환자적자주신경계통손해증상,병대환자진행피부교감반응(SSR)검사,대운동신경원병적자주신경수손정황진행평고.방법 수집래자2009년11월지2011년8월취진우북경협화의원신경과문진적MND병례.기록환자유무자주신경계통수손표현,포괄:피부마목혹소양;출한이상포괄출한감소、랭한혹다한;구간;류연;피온이상;체위성저혈압;동공대소혹대광반사이상;편비;배뇨장애;성공능장애.대MND환자행SSR검사,측정결과삼조아원기전도실적정상표준,계산SSR이상솔급전생리검사여림상표현지간적상관성.결과 공수집MND환자142례.환자중자주신경공능수손적출현솔분별위:피부마목76례(53.5％);피부소양22례(15.5％);출한이상15례(10.6％);구간13례(9.1％);류연3례(2.1％);피온이상21례(14.8％);체위성저혈압3례(2.1％);편비23례(16.2％);배뇨장애14례(9.9％);성공능장애2례(1.4％).142례MND환자중,SSR정상91례(64.1％),이상51례(35.9％).상지SSR이상12례(8.5％),상지잠복기연장2례(1.4％),파폭강저8례(5.6％),파형소실4례(2.8％);하지SSR이상47례(33.1％),하지잠복기연장4례(8.5％),파폭강저31례(21.3％),파형소실15례(10.6％);상하지균이상위7례(4.9％),상하지파형균소실3례(2.1％).MND환자SSR여정상치비교,상지잠복기연장(P＜0.05),상하지파폭균현저강저(균P＜0.01).ALS적각E1-Escorial진단분형지간차이무통계학의의,하지기병조이상솔최고,차이유통계학의의.각자주신경공능손해표현여상、하지SSR파폭、잠복기지간무명현상관성.결론 MND가출현피부、선체분비、심혈관계통、방광직장괄약기등림상자주신경공능이상적표현.부분MND환자출현SSR적이상,표현위잠복기연장화파폭강저.전생리개변여림상자주신경증상지간불구유상관성.하지기병환자적SSR이상솔교고.
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.
