多系统萎缩患者的尿道括约肌和肛门括约肌肌电图对比分析

目的 比较多系统萎缩(MSA)患者尿道括约肌肌电图(US-EMG)和肛门括约肌肌电图(EAS-EMG)变化特征.方法 对拟诊的9例MSA患者行US-EMG和EAS-EMG测定.分别测定运动单位的平均时限、平均波幅、轻收缩波幅、多相波以及大力收缩募集电位共5组数据,并统计分析组间差异.结果 9例MSA患者US-EMG与EAS-EMG两种检查方法均证实7例患者肌电图结果呈神经源性损伤,两种检查结果中平均波幅[( 1063.44±499.92) μV与(634.89±265.07) μV]、多相波[中位数(四分位数间距),分别为0(0,20％)与57％ (28％,63％)]差异有统计学意义(t=2.567,P=0.033;t =2.833,P=0.012).结论 US-EMG和EAS-EMG在MSA诊断中均具有较特异的诊断价值,虽然US-EMG检查实施有一定技巧性和难度,但其轻收缩波幅等指标的异常对于仅有排尿障碍而无便秘的MSA可能具有更为显著的诊断价值.     

多系统萎缩患者的临床表现、MRI、肛门括约肌肌电图分析

目的分析多系统萎缩患者(MSA)临床表现、头部MRI、肛门括约肌肌电图改变特点,探讨它们在MSA诊断的价值。方法按Gilman诊断标准,回顾性分析46例MSA患者的临床资料、头部MRI及肛门括约肌肌电图检查结果。结果符合很可能MSA39例,可能MSA7例,其中MSA-A型24例,主要临床表现为自主神经功能障碍;MSA-C型16例,主要表现为小脑性共济失调;MSA-P型6例,主要表现为锥体外系症状。MRI显示部分MSA-A患者出现大脑皮质萎缩,小脑改变较轻;MSA-C型主要表现为延髓、脑桥、小脑萎缩;MSA-P主要病变在壳核和苍白球,而小脑、脑桥、延髓病变早期可以不明显。部分患者出现脑桥十字征和壳核裂隙征。36例患者做肛门括约肌肌电图检查,全部出现神经源性损害。结论 MSA早期诊断难度大,结合临床表现、头部MRI检查及肛门括约肌肌电图检查,可提高MSA的诊断准确率。     

帕金森病、P型多系统萎缩及进行性核上性麻痹的肛门括约肌肌电图及相关自主神经损害特点

目的 分析帕金森病(PD)、多系统萎缩和进行性核上性麻痹(PSP)的肛门括约肌肌电图(EAS-EMG)及相关自主神经损害特点,进一步探讨EAS-EMG在鉴别诊断方面的价值.方法 回顾性分析2001-2009年12月我院神经科肌电图室进行EAS-EMG检查的562例患者,对其中60例PD患者(男41例、女19例),68例以帕金森症状为主要表现的多系统萎缩(MSA-P)患者(男35例、女33例),13例PSP患者(男10例、女3例)的相关自主神经损害情况及肌电图进行比较.EAS-EMG选取平均时限、多相波比例和卫星电位出现率作为评价指标,比较各参数的组间差异,其中平均时限按照延长的程度分为轻度(10.0～11.9 ms)、中度(12.0～13.9 ms)、重度(≥14.0 ms).结果 在相关自主神经症状中,阳痿、便秘、尿失禁、尿急、尿频在MSA-P组中的出现率[95.8%(23/24)、94.6%(53/56)、87.7%(50/57)、85.7%(42/49)、76.5%(39/51)]明显高于PD组[61.5%(16/26)、62.3%(33/53)、30.6%(15/49)、46.2%(24/52)、45.7%(21/46)]及PSP组[75.0%(3/4)、62.5%(5/8)、50.0%(4/8)、42.9%(3/7)、42.9%(3/7)].PD、MSA-P和PSP的EAS-EMG异常出现率分别为60%(36/60)、94.1%(64/68)和84.6%(11/13).PD、MSA-P和PSP组间平均时限(ms,12.0±1.6、15.4±3.0、13.8±1.8)、多相波(46.2%±19.2%、63.9%±15.8%、51.5%±12.1%)和卫星电位出现率(9.5%±8.3%、26.5%±15.9%、19.2%±12.5%)的差异均有统计学意义(F=31.724、F=17.412、x2=45.335,均P＜0.01).平均时限延长程度PD组:轻度36.7%、中度36.7%、重度11.7%,正常15.0%;MSA-P组:轻度10.3%、中度23.5%、重度66.2%;PSP组:轻度7.7%、中度61.5%、重度30.8%;各组间的差异有统计学意义.结论 EAS-EMG可以用于鉴别PD、MSA-P和PSP的自主神经损害情况.肛门括约肌的损害较常见于MSA-P,且程度较重;较少见于PD,且程度较轻;PSP介于两者之间.平均时限延长程度的分布或许可以提示骶髓Onuf核在3种疾病中受累程度的差异.

Abstract：

Objectives To assess the value of external anal sphincter electromyography (EASEMG) in evaluating the related autonomic dysfunction in Parkinson's disease ( PD), parkinsonism dominant multiple system atrophy (MSA-P) and progressive supranuclear palsy (PSP). Methods From the records of EAS-EMG collected in our lab (total 562 cases), 60 PD (male 41, female 19), 68 MSA-P (male 35,female 33) and 13 PSP (male 10, female 3) were included in the analysis in this study. Mean duration,polyphasic ratio and satellite potential occurrence rate were comparable among the groups. Mean duration prolongation were graded as normal ( ＜ 10.0 ms), mild ( 10.0-11.9 ms), moderate ( 12.0-13.9 ms)and severe ( ≥ 14.0 ms). Results Among all related autonomic symptoms, the occurrence rate of constipation, urinary incontinence, urgency and frequency in patients with MSA-P(95.8% (23/24) ,94.6% (53/56) ,87.7% ( 50/57 ), 85.7% (42/49), 76.5% ( 39/51 ) ) were higher than that of PD ( 61.5%(16/26), 62.3% (33/53), 30.6% (15/49), 46.2% (24/52), 45.7% (21/46)) and PSP (75.0%(3/4) , 62.5% (5/8), 50.0% (4/8), 42.9% (3/7), 42.9% (3/7)). The abnormal rate of EAS-EMG in PD, MSA-P and PSP were 60.0%, 94.2% and 84.6%, accordingly. Mean duration ( PD ( 12.0 ± 1.6)ms, MSA-P (15.4±3.0) ms, PSP (13.8±1.8) ms), polyphasic ratio (PD 46.2% ±19.2%, MSA-P 63.9% ± 15.8%, PSP 51.5% ± 12.1% ) and satellite potential occurrence rate ( PD 9.5% ± 8.3%,MSA-P 26.5% ± 15.9%, PSP 19.2% ± 12.5% ) varied significantly different among the groups ( F =31.724, F = 17.412, x2 =45. 335, all P ＜0.01 ). Severe mean duration prolongation was overwhelming in MSA-P (66.2% ) , compared with mild 10.3% and moderate 23.5%. The predominant prolongation degree was moderate in PSP (61.5%, mild 7.7%, severe 30.8% ), and mild in PD (36.7%, moderate 36.7% ,severe 11.7%, normal 15.0% ). Conclusions EAS-EMG could play a role in evaluating the related autonomic dysfunctions in PD, MSA-P and PSP. The EAS-EMG impairment was severe and frequent in MSA-P, mild and infrequent in PD, moderate in PSP. The spectrum of mean duration prolongation suggested the possibility of Onuf's nucleus involvement in these diseases.     

多系统萎缩20例患者肛门括约肌肌电图特点分析

目的 分析多系统萎缩(MSA)患者肛门括约肌肌电图(EAS—EMG)的特点，探讨EAS—EMG在MSA中的诊断价值。方法 2001年6月至2002年6月问我院神经内科门诊和病房收治的临床诊断MSA共20例，按照诊断指南分为拟诊MSA(12例)和可能MSA(8例)两组，行EAS—EMG检查。观察有无自发电位；运动单位动作电位(MuPs)的平均时限、平均波幅、多相波百分比、平均相数、有无卫星电位；大力收缩时的相型和波幅。比较两组间上述指标的差异，同时分析EAS—EMG与自主神经功能障碍的关系。结果 全组EAS-EMG的异常率为100%，与临床症状的总体符合率为80．0％，拟诊MSA组和可能MSA组分别为91．7％和62．5%。EMG各项指标在两组之间无明显差异。尿便功能障碍患者的平均时限、平均时限超过10ms的百分比和卫星电位出现率明显高于无尿便功能障碍者。性功能障碍患者的平均时限超过10ms的百分比明显高于无性功能障碍者。结论 全部MSA患者的EAS—EMG都有不同程度的异常，自主神经功能障碍，特别是尿便障碍和性功能障碍对EAS—EMG有一定影响。     

肛门括约肌肌电图对多系统萎缩的诊断价值

目的分析肛门括约肌肌电图的改变对多系统萎缩的诊断价值.方法对27例诊断多系统萎缩的患者组和27例非多系统萎缩者为对照组,行肛门括约肌肌电图检查,观察静息时有无自发电位;轻收缩时运动单位的平均时限、平均波幅、多相波百分比、有无卫星电位;大力收缩时的相型和波幅.对2组各参数进行统计分析.结果多系统萎缩患者组25例(92.6%)肛门括约肌肌电图有不同程度的改变,平均时限、平均波幅、多相波百分比、自发电位与对照组比较有显著性差异(P＜0.001).结论肛门括约肌肌电图检查对多系统萎缩的诊断有一定的价值.在怀疑多系统萎缩时该项检查可作为常规的电生理检查方法.     

肛门括约肌肌电图在鉴别诊断多系统萎缩、帕金森病及晚发型脊髓小脑共济失调中的作用

目的 分析多系统萎缩(multipe system atrophy,MSA)、帕金森病(Parkinson's disease,PD)、晚发型脊髓小脑共济失调(1ate-onset spinocerebellar ataxia,LOSCA)患者的肛门括约肌肌电图(external anal sphincter electromyography,EAS-EMG)的差异等电生理改变特点,为临床鉴别诊断提供依据.方法 分别对88例MSA、69例原发性PD、18例LOSCA患者的电生理检查结果进行回顾性分析.结果 EAS-EMG检查中MSA组有84例(95.5%)患者存在不同程度的神经源性受损,表现为运动单位电位(MUPs)平均时限[(12.92±2.59)ms]显著延长、平均波幅[(648.6±251.0)μV]增高、多相波百分比(42.6%±21.2%)增加、异常自发电位与卫星电位多见,与PD[(8.99±0.47)ms、(470.0±91.9)μV、24.2%±11.0%]及LOSCA组[(9.04±0.62)ms、(493.1±113.7)μV、22.0%±12.1%]相比差异有统计学意义(Welch值分别为:94.240、18.093、26.710,均P=0.000).PD及LOSCA组EAS-EMG检查未发现有神经源性受损,也未见自发电位与卫星电位.结论 MSA临床表现可与PD及LOSCA相似,有时容易相互误诊,EAS-EMG检查可提高MSA的正确诊断率,尤其与PD、LOSCA进行鉴别诊断时有较大价值.

Abstract：

Objective To observe the electrophysiological changes, especially in the examination of external anal sphincter electromyography ( EAS-EMG), with those patients diagnosed as multiple system atrophy ( MSA), Parkinson's disease (PD) and late-onset spinocerebellar ataxia (LOSCA) and explore its clinical diagnostic value as well as differential diagnostic value for the three diseases. Methods The clinical data, cranial magnetic resonance imaging (MRI) data as well as results of EAS-EMG for 3 groups patients, including 88-cases MSA, 69-cases PD and 18-cases LOSCA, were analyzed retrospectively.Results EAS-EMG showed that 84 cases (95.5%) in MSA group had varying degrees of neurogenic injury. Meanwhile, mean motor unit potentials (MUPs) duration (( 12.92 ± 2.59)ms), mean MUPs amplitude ( ( 648.6 ± 251.0 ) μV ), and MUPs polyphasicity ( percentage of polyphasic MUPs; 42. 6% ±21.2% ) in MSA group were significantly different from those in PD ( ( 8. 99 ± 0. 47 ) ms, (470. 0 ±91.9) μV, 24.2% ±11.0%) and LOSCA groups ((9.04 ±0.62)ms, (493.1 ± 113.7)μV,22.0% ±12. 1%; Welch:94. 240,18. 093,26. 710,all P =0. 000). The spontaneous potentials and satellite potentials showed more common in MSA group, but not in other groups. Conclusions MSA and PD and LOSCA are easily mutually misdiagnosed because of some similar syndromes, but the method of EAS-EMG could be effective and helpful to enhance accurate diagnostic rate of MSA and its differential diagnosis with PD and LOSCA.     

肛门括约肌肌电图对多系统萎缩诊断价值的研究进展

多系统萎缩(MSA)是一种少见的,散发的慢性神经系统变性疾病。MSA出现骶髓前角选择性的细胞脱失,同时伴有残留运动神经元的侧枝芽生。肛门括约肌肌电图(EAS-EMG)表现为时限延长、多相波增多、出现自发电位或卫星电位等神经源性损害的改变。在怀疑MSA时该项检查可作为常规的电生理检查方法。本文就近年EAS-EMG在MSA诊断中的应用作一综述。     

肛门括约肌肌电图对多系统萎缩Onuf核损伤的判断

目的分析多系统萎缩的肛门括约肌肌电图特点,探讨其在多系统萎缩Onuf核损伤判断中的价值。方法多系统萎缩患者68例和肌萎缩侧索硬化15例,分别行肛门括约肌肌电图检查,观察静息时有无自发电位;轻收缩(自然状态)时运动单位电位的平均时限、平均波幅、多相波百分比、有无卫星电位;大力收缩(缩肛)时的相型和波幅。对两组各参数进行统计分析。结果多系统萎缩患者组65例(95.6%)肛门括约肌肌电图有不同程度的改变,肌萎缩侧索硬化组未发现异常变化。运动单位电位的平均时限、平均波幅、多相波百分比、自发电位两组比较有显著性差异(P<0.001,P<0.01,P<0.001)。结论多系统萎缩肛门括约肌肌电图的改变,能反映多系统萎缩的Onuf核选择性的弥漫性细胞脱失的特性。在怀疑多系统萎缩Onuf核损伤时,该项检查对损伤的判断有较大价值。     

肛门括约肌肌电图及其在多系统萎缩等神经系统疾病中的诊断价值

1978年 ,Ｓａｋｕｔａ等报道了肛门括约肌肌电图 (ｅｘｔｅｒｎａｌａｎａｌｓｐｈｉｎｃｔｅｒＥＭＧ ,ＥＡＳ ＥＭＧ)用以区分Ｓｈｙ Ｄｒａｇｅｒ综合征和肌萎缩侧索硬化 (ＡＬＳ) [1] ,并首次将此项操作技术应用于临床。此后的 2 0多年里 ,越来越多的神经科学者发现了ＥＡＳ ＥＭＧ在多系统萎缩 (ＭＳＡ)、帕金森病 (ＰＤ)等神经系统变性病中的诊断和鉴别诊断价值 ,为临床科研工作提供了极大的帮助。一、ＥＡＳ ＥＭＧ的解剖[2 ] 及生理基础肛门外括约肌是环绕肛管的椭圆柱形骨骼肌 ,肌纤维细小 ,Ⅰ型和Ⅱ型纤维直径分别为 2 0、40 μｍ…     

多系统萎缩患者的临床、影像及电生理分析

目的 探讨多系统萎缩(multiple system atrophy,MSA)的临床、影像以及电生理改变特点,为临床诊断提供依据.方法 对62例MSA患者的临床资料、神经影像学以及肌电图检查结果进行回顾性分析.结果 我们共收集拟诊MSA患者62例,其中MSA-A型29例(46.8 % ),主要临床特点表现为直立性低血压为主的自主神经系统症状;MSA-C型24例(38.7 % ),主要表现为小脑性共济失调;MSA-P型9例(14.5 % ),则以锥体外系症状为主.头颅MRI显示MSA-A型患者部分出现小脑病灶;MSA-C型患者主要病变在小脑、脑桥和延髓;MSA-P型患者病变主要在壳核.51例患者行肛门括约肌肌电图(external anal sphincter electromyography,EAS-EMG)检查,其中46例示典型神经源性损害;19例(30.6 % )曾被误诊为其他疾病.结论 MSA早期易漏诊或误诊,结合临床表现、神经影像学以及EAS-EMG检查,可提高MSA的诊断率.     

The nature of the autonomic dysfunction in multiple system atrophy

The concept that multiple system atrophy (MSA, Shy-Drager syndrome) is a disorder of the autonomic nervous system is several decades old. While there has been renewed interest in the movement disorder associated with MSA, two recent consensus statements confirm the centrality of the autonomic disorder to the diagnosis. Here, we reexamine the autonomic pathophysiology in MSA. Whereas MSA is often thought of as “autonomic failure”, new evidence indicates substantial persistence of functioning sympathetic and parasympathetic nerves even in clinically advanced disease. These findings help explain some of the previously poorly understood features of MSA. Recognition that MSA entails persistent, constitutive autonomic tone requires a significant revision of our concepts of its diagnosis and therapy. We will review recent evidence bearing on autonomic tone in MSA and discuss their therapeutic implications, particularly in terms of the possible development of a bionic baroreflex for better control of blood pressure.     

The assessment of sudomotor dysfunction in multiple system atrophy

We studied sudomotor function in 21 patients with multiple system atrophy and in 11 age-matched controls. The extent and severity of the sudomotor deficit was assessed using the quantitative thermoregulatory sweat test. Central sudomotor function was studied by measuring sweating in response to raising body heat and administering thyrotropin-releasing hormone. Postganglionic sudomotor function was studied using the sudomotor axon reflex test evoked by nicotine. We conclude that in multiple system atrophy, thermoregulatory sudomotor dysfunction was more severe in the lower extremities. Heat stimulation increased the frequency of sweat expulsion and sweat rate on the forearm in moderate multiple system atrophy to a similar degree as controls but failed to do so on the thigh. Thyrotropin-releasing hormone enhanced sweating in moderate multiple system atrophy and controls. Results of the sudomotor axon reflex test indicate that in multiple system atrophy there is postganglionic sudomotor dysfunction which may be due to transsynaptic changes. These results suggest that the main lesion responsible for sudomotor dysfunction in multiple system atrophy is within the intermediolateral column cells of the spinal cord.     

Metaiodobenzylguanidine (MIBG) scintigraphy at various parts of the body in Parkinson's disease and multiple system atrophy

We compared MIBG uptake at various parts of the body in controls and patients with Parkinson's disease and multiple system atrophy. In the heart, MIBG uptake in Parkinson's disease (early H/M: 1.668+/-0.325, late H/M: 1.500+/-0.402) was less than that in multiple system atrophy (early H/M: 2.395+/-0.186, late H/M: 2.530+/-0.391) and controls (early H/M: 2.635+/-0.508, late H/M: 2.575+/-0.635) (early: P<0.0001, late: P<0.0001). There were no significant differences in uptake by the lung, thyroid, or liver in the three groups. Only on early images, uptake in the shoulder in multiple system atrophy (early S/M: 0.473+/-0.78) and Parkinson's disease (early S/M: 0.470+/-0.710) was decreased compared to that in controls (early S/M: 0.560+/-0.118) (P=0.0252). MIBG is reported to be taken up in the terminal part of sympathetic nerves and demonstrates sympathetic nerve activity, especially on late images. The cause of differences between the heart and other parts of the body remains unknown. We consider the following possibilities: (a) differences in the sympathetic nervous system between Parkinson's disease and multiple system atrophy are more subtle in organs other than the heart; (b) the cause of MIBG uptake reduction by the heart in Parkinson's disease involves factors in addition to sympathetic nervous system damage; and (c) MIBG uptake by organs other than the heart involves not only the sympathetic nervous system but also non-neuronal components. In conclusion, MIBG uptake by organs other than the heart cannot differentiate Parkinson's disease from multiple system atrophy at present.