肌萎缩侧索硬化与脊髓型颈椎病的神经电生理检查

目的 探讨神经电生理检查在肌萎缩侧索硬化（amyotrophic laleral sclerosis，ALS）及脊髓型颈椎病（cervical spondylotic myelopathty，CSM）鉴别中的价值。方法 对28例初步诊断为ALS组、34例CSM组及两者鉴别困难者5例，这三组病人进行肌电图检查。结果 ALS组胸锁乳突肌及上、下肢肌肌电图都表现为神经源性损害；而CSM组仅表现为上肢脊神经根所支配的相应区域的神经源性损害，胸锁乳突肌及下肢肌无一例异常；而两者鉴别困难者组通过肌电图检查发现有4例存在颈、腰椎神经根病变，有1例为颈椎病合并ALS。本文同时对下胸段脊旁肌（T9．T10、T11）进行检查发现AIS组中阳性率为75％，而颈椎病组无一例异常。结论 神经电生理检查能帮助ALS及CSM的诊断及鉴别诊断。     

肌萎缩侧索硬化症152例下胸段脊旁肌肌电图特点

目的探讨下胸段脊旁肌肌电图在肌萎缩侧索硬化症(ALS)诊断中的应用价值。方法总结152例确诊ALS患者的临床及电生理资料,观察下胸段脊旁肌肌电图的特点,并分析其与病程、年龄、首发部位、呼吸困难、胸锁乳突肌肌电图、舌肌肌电图的相关性。结果152例ALS中,下胸段脊旁肌肌电图出现自发电位者125例(82．24%)；胸锁乳突肌肌电图呈神经源性损害83例(55．33%),其中出现自发电位者45例。通过Logistic多元回归分析提示脊旁肌肌电图与病程、年龄有关,而与首发部位、呼吸困难无明显相关性。结论脊旁肌肌电图与ALS患者的病程及年龄有关,且下胸段脊旁肌肌电图方便易行,在ALS中诊断价值较大。     

139例肌萎缩侧索硬化临床及肌电图表现特点

目的 探讨肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)的临床及电生理特征,为早期准确诊断ALS提供依据.方法 回顾性研究近5年来收治的门诊及住院139例肌萎缩侧索硬化患者的临床及电生理表现,对其发病特点、症状、体征及实验室检查进行统计分析.结果 ALS在临床上慢性隐袭起病,逐渐进展,50岁前后发病,平均年龄49.1岁,平均病程2.4年.男性明显多于女性.首发症状为单侧上肢肌肉无力和肌肉萎缩最常见,其次为肌束震颤、延髓麻痹、锥体束征等,少数患者可出现肢体麻木、疼痛或发凉等感觉异常.临床主要症状是肢体无力、肢体和舌肌肌肉萎缩、锥体束征、肢体和舌肌肌束震颤.所有ALS侵害的部位均表现神经源性损害,胸锁乳突肌肌电图检查、胸脊旁肌肌电图、头部/颈/腰椎CT及MRI等辅助检查具有重要的确诊及鉴别诊断意义.结论 目前ALS的诊断仍然依靠临床表现.肌电图、CT/MRI是ALS诊断和鉴别诊断的重要辅助手段.     

胸段脊旁肌肌电图在肌萎缩侧索硬化诊断中的作用

目的 研究胸段脊旁肌自发电位在诊断肌萎缩侧索硬化(amyotrophic lateral sclerosis，ALS)中的价值。方法 50例确诊的ALS患者分别行胸段脊旁肌自发电位、胸锁乳突肌运动单位，部分患者行舌肌自发电位检测；同时，将性别、年龄相匹配的30例根性损害的患者和30名健康人作为对照，行胸段脊旁肌自发电位检测。结果 50例ALS患者中，41例(82％)胸段脊旁肌肌电图可见大量纤颤电位和正锐波；胸锁乳突肌肌电图无一例见自发电位，但有48例(96%)运动单位时限增宽，波幅增高，符合慢性神经源性损害改变；6例患者行舌肌自发电位检测，3例可见自发电位。30例根性病变的患者中，2例(7％)可见胸段脊旁肌有少量自发电位；健康对照组未见胸段脊旁肌自发电位。结论 胸段脊旁肌大量自发电位对诊断ALS具有一定的敏感性。     

肌萎缩侧索硬化与脊髓型颈椎病的鉴别诊断

目的　探讨肌萎缩侧索硬化 (ALS)与脊髓型颈椎病 (CSM )早期临床鉴别诊断方法。方法　回顾性对临床确诊的 36例ALS和 2 2例CSM主要症状 /体征、辅助检查异常率进行比较。结果　除肌无力为两病共同的常见症状和体征外 ,肌萎缩、肌束震颤、感觉异常、病理征阳性率皆有显著性差别 ;ALS不存在客观感觉障碍 ,CSM不存在构音 /吞咽障碍和掌颏反射阳性。运动单位减少 ,波幅增高 ,时限增宽 ,纤颤电位增多 ,感觉传导速度和体感诱发电位正常 ,胸锁乳突肌EMG异常均利于ALS诊断。结论　在临床早期 ,通过识别关键性的症状、体征 ,结合电生理等实验室检查 ,可以对ALS和CSM作出正确的鉴别诊断     

肌萎缩侧索硬化症90例胸锁乳突肌肌电图的特点

目的　探讨胸锁乳突肌肌电图 (EMG)检测在肌萎缩侧索硬化症 (ALS)诊断中的价值。方法　对ALS患者均进行常规EMG检测 ,包括上肢、下肢及胸锁乳突肌 ,测定自发电位 ,募集相 ,运动单位电位的时限、波幅及多相波。比较有、无球部症状的患者之间胸锁乳突肌EMG改变的差异 ,以及不同部位即球部、颈部及腰骶部之间EMG检测结果的差异。结果　有球部症状的患者胸锁乳突肌EMG病变程度重于无球部症状者 (时限增宽幅度分别为 60 6 %± 2 1 2 %和 50 0 %± 1 9 2 % ,P <0 0 5)。胸锁乳突肌EMG自发电位、募集相、运动单位电位时限和波幅改变的程度均不同于上肢和下肢 ,存在显著性差异。结论　胸锁乳突肌EMG检测的异常与球部症状相关 ,而与肢体无关 ,其神经源性改变可反映球部下运动神经元病变     

不同起病部位的ALS病人异常肌电区域分布研究

目的 :探讨不同起病部位的ALS病人在 4个下运动神经元支配区域异常肌电的分布情况。方法 :对 10 2例临床诊断为ALS的病人分别行 4组下运动神经元区域 (脑干 ,颈 ,胸 ,腰骶髓 )的针极肌电图检查。对 87例同时进行了胸锁乳突肌的检查。结果 :4组肌电检查区域的结果示 :颈段与胸段异常率在各起病组中最高 ,脑干及腰骶段在各组中异常率不同 (差异有显著意义 ,P <0 0 5 ) ;胸锁乳突肌上、下肢起病组的异常率均高于同组的脑干支配肌。结论 :在ALS病人中 ,异常肌电的分布与起病部位有关。不论哪一部位起病 ,4个区域均可受累 ,因此必须全都检查。胸锁乳突肌具有特殊的诊断及鉴别诊断价值     

运动神经元病临床与骨骼肌病理112例诊断分析

目的探讨骨骼肌病理对运动神经元病诊断与鉴别诊断的价值。方法收集112例运动神经元病患者的临床、神经电生理及活检骨骼肌病理资料,进行诊断与鉴别诊断分析。结果①入选患者均有肢体无力、肌萎缩,吞咽或呼吸肌无力43例,舌肌萎缩或纤颤50例,伴有肌束震颤69例,初诊伴有上运动神经元受损征78例;②肌电图呈神经源性异常;③临床确诊肌萎缩侧索硬化(ALS)90例(初诊78例、复诊/随访后12例),脊髓性肌萎缩症(SMA)22例,其中肯尼迪病7例;④骨骼肌病理均符合神经源肌病改变,ALS多见小角化肌纤维、核聚集、靶纤维;成人型SMA以小圆形肌纤维在肌束内小群分布为特点,其中肯尼迪病萎缩小圆形肌纤维、核聚集多在肌束间分布。结论①仅表现下运动神经元受累的MND,行骨骼肌活检病理分析有助ALS与SMA的诊断与鉴别诊断;②随诊、动态观察体征与病情进展变化可助ALS确诊。     

平山病的神经电生理学研究

目的 探讨平山病的神经电生理学特点及其与肌萎缩侧索硬化、多灶性运动神经病的鉴别诊断.方法 分别对平山病(26例)、肌萎缩侧索硬化(30例)和多灶性运动神经病(16例)患者进行运动和感觉传导速度、肌电图及交感皮肤反应等神经电生理学检查.运动传导速度采用由远端至近端分段刺激,记录复合肌肉动作电位的波幅、时限、面积及波形的变化,并判断是否存在神经传导阻滞;肌电图检查记录脑区肌肉(双侧胸锁乳突肌),颈区肌肉(拇短展肌、小指展肌、第一骨问肌、肱二头肌),胸区肌肉(T10椎旁肌、腹直肌)和腰骶区肌肉(胫骨前肌)的肌电活动.比较3组患者神经电生理学特点的差异性.结果 平山病组患者均无神经传导阻滞,肌电图检查显示颈区肌肉呈神经源性损害;肌萎缩侧索硬化组患者亦无神经传导阻滞,肌电图检查显示脑区、颈区、胸区和腰骶区肌肉均呈神经源性损害;多灶性运动神经病组患者均存在神经传导阻滞,肌电图检查颈区和腰骶区肌肉呈神经源性损害.平山病组患者神经传导阻滞的发生率与多灶性运动神经病组比较,差异有统计学意义(x2=42.000,P=0.000);平山病组患者神经源性损害的发生率与肌萎缩侧索硬化组比较,差异亦有统计学意义(x2=56.000,P=0.000).结论 平山病组患者运动和感觉传导速度均无异常,无神经传导阻滞,但肌电图检查显示颈区肌肉呈神经源性损害.     

Kennedy病患者电生理、病理学特点及临床误诊分析

目的研究肯尼迪病(Kennedy disease,KD)患者电生理学特征和肌肉病理学特点,探讨临床误诊原因,以加强对此病电生理学改变和肌肉病理学的认识,减少误诊误治。方法对9例经基因确诊的KD患者行针极肌电图、神经传导、F波和H反射检测。肌电图取舌肌、胸锁乳突肌、三角肌、第一骨间肌、胸椎旁肌、股四头肌、胫前肌。神经电图取双侧正中神经、尺神经、腓总神经、腓肠神经和胫神经。1例患者取股四头肌肌肉活检行病理学检查。结果肌电图检查显示9例肯尼迪病患者均表现为广泛的慢性神经源性损害。CMAP和SNAP波幅均显著降低(P0.01),以腓肠神经动作电位波幅异常率最高(88.89%),腓总神经和腓肠神经传导速度减慢(P0.01),正中神经和胫神经远端潜伏期延长(P0.01),正中神经和胫神经F波响应率降低,胫神经的H反射潜伏期延长(P0.01)。肌活检示神经源性肌损害,表现为肌纤维萎缩,萎缩纤维群组化分布。结论 KD患者电生理以广泛的慢性神经源性损害为最主要改变,感觉和运动神经传导异常,且感觉神经受累较运动神经受累更多见,病理学表现为肌肉萎缩及群组化分布。电生理和肌肉活检对该病的早期诊断和减少误诊率有重要指导价值。     

Electromyography of sternocleidomastoid muscle in ALS: a prospective study

Needle electromyography (EMG) of the tongue is difficult to perform because of frequent uncontrollable movement. We chose the sternocleidomastoid (SCM) muscle as a possible alternative for assessing the involvement of the rostral neuraxis in amyotrophic lateral sclerosis (ALS). We prospectively studied 21 ALS patients during our initial diagnostic evaluation. EMG parameters that we recorded included the presence of abnormal spontaneous activity, pattern of motor unit potential recruitment, and configuration of motor unit action potentials. For the six patients with bulbar-onset ALS, three had abnormalities in the SCM and three had abnormalities in the tongue. In contrast, for the 15 patients with limb-onset ALS, 9 had abnormalities in the SCM, and only 3 had abnormalities in the tongue. Our results demonstrate the utility of needle EMG of the SCM in the evaluation of ALS. EMG of the SCM carries a similar sensitivity as the tongue in ALS patients with bulbar symptoms, but is more sensitive than the tongue in patients without bulbar symptoms. SCM innervation includes the rostral cervical cord and brainstem, and EMG abnormalities in this muscle support a diffuse involvement, which is unique to ALS.     

Difference in distribution of fasciculations between multifocal motor neuropathy and amyotrophic lateral sclerosis

ObjectiveTo examine differences in fasciculation distribution between patients with multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS) based on muscle ultrasound.MethodsForty-one muscles (tongue muscle and 40 muscles of the trunk and limbs on both sides) in 5 MMN patients and 21 muscles (tongue muscle and 20 muscles on the onset side) in 21 ALS patients were subjected to muscle ultrasound individually for 60 seconds to detect the presence of fasciculations.ResultsFasciculation detection rates on the onset side were significantly higher in ALS (42.4 ± 18.3%, mean ± SD) than in MMN (21.9 ± 8.8%) patients (p < 0.05). In MMN patients, no fasciculation was detected in the tongue or truncal muscles. There was no difference in the fasciculation detection rate between the onset and non-onset sides or between upper and lower limbs in MMN patients.ConclusionsIn MMN patients, fasciculations were detected extensively in the limbs. However, the detection rate in patients with MMN was lower than in those with ALS.SignificanceDemonstration of the absence of fasciculations in the tongue and truncal muscles in MMN patients by extensive muscle ultrasound examination may help distinguish MMN from ALS.     

Amyotrophic lateral sclerosis versus cervical spondylotic myelopathy: a study using transcranial magnetic stimulation with recordings from the trapezius and limb muscles.

OBJECTIVE: We report an electrophysiological method to differentiate amyotrophic lateral sclerosis (ALS) from cervical spondylotic myelopathy (CSM). METHODS: Motor evoked potentials (MEPs) by transcranial magnetic stimulation were investigated in patients with ALS (n=10) and CSM (n=9). In addition to limb MEPs using the triple stimulation technique (TST) at upper limbs, MEPs recorded from trapezius muscles were compared with those obtained from 23 normal subjects. The parameters studied were: central motor conduction time, amplitude ratio and, for the trapezius, the interside asymmetry. RESULTS: Whereas limb MEPs were abnormal in most ALS and CSM patients (17/19), trapezius MEPs were abnormal in all ALS patients, and normal in 8 out of 9 CSM patients. CONCLUSION: Recording of trapezius MEPs is a valuable addition to the limb MEPs study, since it distinguishes ALS from SCM in most patients.     

Needle electromyography of the frontalis muscle in patients with amyotrophic lateral sclerosis

Introduction: We sought to determine which muscles to choose for better assessment of the craniobulbar region in establishing the diagnosis of amyotrophic lateral sclerosis (ALS). Methods: We studied the frontalis muscle in 83 controls and compared it with the tongue, sternocleidomastoid (SCM), and trapezius muscles in 105 definite or probable ALS patients (54 bulbar, 51 nonbulbar). Results: More patients achieved complete relaxation of the frontalis muscle than the tongue or SCM. Motor unit potentials were of longer duration and higher amplitude in ALS patients than in controls (P < 0.05). The frontalis had the same frequency of spontaneous potentials as the tongue, SCM, and trapezius muscles in bulbar ALS patients, but fewer than in the trapezius in nonbulbar patients. Conclusions: Examining the frontalis provides useful information in establishing the diagnosis of ALS by identifying clinically evident or subclinical abnormalities in the craniobulbar region. Muscle Nerve 54 : 1093–1096, 2016     

上肢DSSEPs对ALS与CSM鉴别诊断的研究

以上肢皮节体感诱发电位(DSSEPs)鉴别肌萎缩侧索硬化(ALS)与脊髓型颈椎病(CSM),结果发现前者阳性率8％,后者阳性率100％,二者存在极显著性差异(P<0.001)。进一步将异常结果与病人颈部MR对照分析,发现两方法对颈部病损定位诊断间存在线性关系。结果提示:此检查有助于ALS与CSM的鉴别。     

Utility of trapezius EMG for diagnosis of amyotrophic lateral sclerosis

Needle electromyography (EMG) of the tongue is traditionally used as a key to the diagnosis of amyotrophic lateral sclerosis (ALS), although relaxation of the tongue is often difficult to achieve. Recently, frequent abnormalities in the EMGs of the sternocleidomastoid (SCM) and upper trapezius muscles in ALS have been reported. To elucidate the diagnostic utility of these muscles we performed a multicenter prospective study to examine EMGs of the tongue (genioglossus), SCM, and trapezius in 104 ALS or suspected ALS patients. We also examined EMGs of the SCM and trapezius in 32 cervical spondylosis (CS) patients. We mainly evaluated fibrillation potentials/positive sharp waves (Fib/PSWs) and fasciculation potentials. Complete relaxation was achieved in 85% of ALS patients in the trapezius, but in only 6% of patients in the tongue. Fib/PSWs were observed in 8%, 13%, and 45% of ALS patients in the tongue, SCM, and trapezius, respectively, whereas fasciculation potentials were observed in 1%, 7%, and 39%, respectively. Abnormal spontaneous activity of any type was found in 9%, 17%, and 63% of patients, respectively. The high frequency of abnormal spontaneous activity in the trapezius was similar among the different diagnostic categories, and even 72% of clinically suspected ALS (progressive muscular atrophy) patients showed them in their trapezius. We did not observe Fib/PSWs or fasciculation potentials in any of our CS patients, thus these findings have excellent specificity. Tongue EMG added little utility over the clinical sign of tongue atrophy. Abnormal spontaneous activity in the trapezius would be more useful for the early diagnosis of ALS. Muscle Nerve 39: 63–70, 2009     

斜方肌肌电图在肌萎缩侧索硬化早期诊断中的应用

目的 探讨斜方肌肌电图在检测肌萎缩侧索硬化(ALS)延髓部下运动神经元损害中的价值.方法 对100例ALS患者、80例颈椎病患者和100名健康志愿者进行斜方肌肌电图检测,AKS组和颈椎病组同时进行胸锁乳突肌、第一骨间肌、腹直肌、胫前肌肌电图检测,比较3组肌电图的差异.颈椎病组中43例患者进行手术前后斜方肌肌电图比较.结果 ALS组中,病程≤8个月的患者斜方肌肌电图自发电位的出现率高于病程>8个月者[分别为21/30(70%)和28/70(40%),X~2=7.56,P=0.004];斜方肌肌电图与胸锁乳突肌肌电图异常率比较差异无统计学意义.ALS组[波幅(1086.9±152.6)μV,时限(17.2±6.5)ms,多相波23.6%±3.4%]与对照组[波幅(606.7±82.7)μV,时限(11.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±2.0)ms,多相波13.5%±2.4%]间运动单位动作电位(MUAP)各参数比较,差异有统计学意义(q=8.32、4.25、4.23,均P<0.01).颈椎病患者手术前后斜方肌肌电图MUAP各参数无明显变化,5例术后发现少量自发电位.结论 斜方肌肌电图可作为检测ALS延髓部下运动神经元损害的辅助手段,特别是早期可见自发电位增多. 1.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±2.0)ms,多相波13.5%±2.4%]间运动单位动作电位(MUAP)各参数比较,差异有统计学意义(q=8.32、4.25、4.23,均P< .01).颈椎病患者手术前后斜方肌肌电图MUAP各参数无明显变化,5例术后发现少量自发电位.结论 斜方肌肌电图可作为检测ALS延髓部下运动神经元损害的辅助手段,特别是早期可见自发电位增多. 1.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±     

Spontaneous electromyographic activity of the tongue in amyotrophic lateral sclerosis

Introduction: Detection of denervation in muscles in the craniobulbar area is important to assure widespread lower motor neuron involvement in the diagnosis of amyotrophic lateral sclerosis (ALS). The value of spontaneous activity analysis in needle electromyography (EMG) of the tongue has been questioned in the recent literature. Methods: Spontaneous activity in the tongue and sternocleidomastoid (SCM) muscles was reviewed retrospectively in 17 ALS patients. Results: Needle EMG showed spontaneous activity in the tongue in 14 of 17 patients (82%) and in 6 patients of 17 (35%) in SCM. Spontaneous EMG activity in the tongue was found in patients with and without bulbar symptoms. Conclusions: Needle EMG is a valuable method for assessing clinical and subclinical involvement of the tongue in patients with bulbar and limb onset ALS. Adequate relaxation of the tongue is a prerequisite for proper spontaneous activity recording. Muscle Nerve, 48: 296–298, 2013     

Motor responses of the sternocleidomastoid muscle in patients with amyotrophic lateral sclerosis

The sternocleidomastoid muscle (SCM) is an accessory inspiratory muscle, but it is not investigated systematically in patients with amyotrophic lateral sclerosis (ALS). We aimed to study the involvement of the SCM and to evaluate the role of the diaphragm and SCM on respiratory tests performed in ALS patients. We studied 45 patients (mean age +/- SD: 60.6 +/- 13 years). In all patients we evaluated: neck flexion strength; forced vital capacity (FVC); maximal inspiratory pressure (PImax); sniff nasal inspiratory pressure (SNIP); ALS functional scale (ALS-FRS-R); SCM and diaphragm compound muscle action potential (CMAP) amplitudes (SCM Ampl and Diaphr Ampl) and latencies (SCM Lat and Diaphr Lat). In ALS patients, SCM Lat is increased and SCM Ampl is smaller in patients with neck weakness. The subgroup of patients with neck weakness had more abnormal respiratory function tests and lower clinical scores. There is a significant correlation between SCM Amp and Diaphr Ampl, FVC, PImax, and SNIP. Hence, there is a parallel loss of motor units in the SCM and diaphragm. On multiple regression analysis both PImax and SNIP are dependent on SCM Ampl and Diaphr Ampl, but FVC is not. PImax and SNIP determination mostly depend on SCM and diaphragm function, but the FVC is also dependent on expiratory muscle function. We conclude that neck weakness is a clinical sign that indicates a poor prognosis, and the SCM CMAP can contribute to respiratory function evaluation in ALS patients.     

Electrical impedance myography in the evaluation of the tongue musculature in amyotrophic lateral sclerosis

Introduction: Electrical impedance myography (EIM) quantifies muscle health and is used as a biomarker of muscle abnormalities in neurogenic and myopathic diseases. EIM has yet to be evaluated in the tongue musculature in patients with amyotrophic lateral sclerosis (ALS), who often show clinical bulbar signs. Methods: The lingual musculature of 19 subjects with motor neuron disease and 21 normal participants was assessed using EIM, strength and endurance testing, and clinical assessment. Results: Tongue musculature in the ALS group was characterized by significantly smaller phase (Ph) and greater resistance (R) when compared with the healthy cohort. Ph and tongue endurance were correlated in the ALS group. Conclusions: EIM of tongue musculature could distinguish those with ALS from healthy controls. The demonstrated relationship between tongue function and Ph supports further testing of EIM of the tongue as a potential biomarker in ALS. Muscle Nerve 52: 584–591, 2015