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。     

中国肌萎缩侧索硬化诊断和治疗指南

运动神经元病是一种病因未明、主要累及大脑皮质、脑干和脊髓运动神经元的神经系统变性疾病,包括肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)、进行性肌萎缩、进行性延髓麻痹和原发性侧索硬化4种临床类型.ALS是运动神经元病中最常见的类型,一般中老年发病多见,以进行性加重的骨骼肌无力、萎缩、肌束颤动、延髓麻痹和锥体束征为主要临床表现,生存期通常3～5年. ALS的早期临床表现多样,缺乏特异的生物学确诊指标.详细的病史、细致的体检和规范的神经电生理检查对于早期诊断具有关键性的作用,影像学等其他辅助检查在鉴别诊断中具有一定价值.临床诊断过程中,确定上、下运动神经元受累范围是诊断的关键步骤,根据患者所出现症状、体征的解剖部位,通常将受累范围分为脑干、颈段、胸段和腰骶段4个区域.     

胸锁乳突肌肌电图在鉴别肌萎缩侧索硬化与颈椎病性脊髓病的研究

对106例肌萎缩侧索硬化(ALS)与颈椎病性脊髓病(CSM),及两病鉴别困难者进行胸锁乳突肌、肢体肌及舌肌EMG检查。结果ALS组胸锁乳突肌神经源性损害的异常率高于三肢体肌、舌肌;CSM组胸锁乳突肌无1例异常。表明该肌神经源性损害能明显提高ALS亚临床的阳性率,有助于ALS的早期诊断及ALS与CSM两病的鉴别。     

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。     

三叉神经-颈反射对运动神经元病球部损害的诊断价值

目的 建立三又神经-颈反射(TCR)的肌电检测方法,探讨其对运动神经元病(MND)球部损害的诊断价值。方法受检测者取仰卧位,头部轻度抬高。刺激一侧眶下神经,于双侧胸锁乳突肌记录。结果 刺激对照者一侧的眶下神经,可于双侧胸锁乳突肌引出正/负波。MND组:7例正常(23.3％),8例未引出(26.7％),11例潜伏期延长(36.7％),4例双侧反射超常不对称(13.3％)。结论TCR能够可靠测定,作为颈-球区病变的一种辅助检查手段,有助于运动神经元病的早期诊断。     

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     

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.     

上运动神经元损害为主的肌萎缩侧索硬化患者的临床和神经电生理特点

目的 探讨以上运动神经元损害为主要表现的肌萎缩侧索硬化( UMN-D ALS)的临床和神经电生理特点.方法 回顾分析76例UMN-D ALS患者及19例原发性侧索硬化(PLS)患者,对其临床表现和神经电生理特点进行总结、比较.神经电生理研究主要包括四肢神经传导速度和延髓、颈、胸、腰骶4个区的肌肉肌电图检测,每隔6个月复查1次.结果 8例初诊为PLS的患者随访中出现下运动神经元损害的表现,转入UMN-D ALS组,此组患者增为84例.＞40岁的UMN-D ALS患者中女性更多(男:女=1∶1.37).32例(38.1％)延髓部起病,从首发症状到肌电图提示神经源性改变平均为30个月,77例(91.6％)在病程4年内出现下运动神经元损害的表现.随访4年时,UMN-D ALS组修改版ALS神经功能评分(分)由40±3下降为32±4(t=1.83,P＜0.05);UMN-D ALS组与PLS组第一骨间肌运动单位动作电位波幅、时限相比[(1003.7±25.2) μV和(353.5±21.5) μV,t=2.34,P＜0.05;(19.8±2.3)ms和(9.6±1.3)ms,t=1.85,P＜0.05]差异有统计学意义.结论 UMN-D ALS患者中女性、以延髓部起病患者比例较高,比PLS进展快,肌电图神经源性损害局限.     

肌萎缩侧索硬化症的临床特点与起病部位的关系研究

目的:探索肌萎缩侧索硬化症(ALS)发病的危险因素与起病部位的关系,为该病的早期诊断提供新思路.方法:选取复旦大学附属华山医院神经内科2000至2010年出院诊断为ALS患者57例,回顾性分析患者的临床资料及相关危险因素.根据不同的起病部位分为延髓起病组、颈段起病组、腰段起病组.通过单因素/多因素回归方法分析比较各组间...     

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.     

The electromyographic diagnosis of amyotrophic lateral sclerosis: Does the evidence support the El Escorial criteria?

The aim of this retrospective cohort study was to examine the accuracy of the El Escorial electromyographic criteria for the diagnosis of amyotrophic lateral sclerosis (ALS). Based on a consecutive series of 73 patients with suspected ALS, the sensitivity of electromyography (EMG) ranged from 0.2 in the cranial segment to 0.74 in the thoracic segment. Specificity was highest (0.92-1.0) in the cranial and thoracic segments. Using receiver operating characteristic (ROC) curve analysis to examine the effect of varying the number of abnormal segments required for the diagnosis, we found an overall accuracy of 0.90 and 0.87 if one or two abnormal segments, respectively, were required. The best combination of sensitivity and specificity was achieved by requiring EMG changes in two segments with abnormalities in a single muscle in the cranial and thoracic segments, but abnormalities in two muscles in the cervical and lumbosacral regions. These findings lend support to the EMG criteria proposed at El Escorial.     

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。     

Prognostic value of EMG genioglossus involvement in amyotrophic lateral sclerosis

ObjectiveTo evaluate the prognostic value of needle electromyography (EMG) genioglossus involvement in patients with amyotrophic lateral sclerosis (ALS) at diagnosis.MethodsWe separately explored the prognostic value of clinical bulbar lower motor neuron (LMN) signs and EMG genioglossus involvement using Cox proportional hazard models adjusted for age, gender, diagnostic delay, presence of bulbar upper motor neuron (UMN) signs, EMG cervical and lumbosacral region involvement, ALSFRS-R score and C9Orf72 gene status. Then, we compared the prognostic value of EMG masseter and genioglossus abnormalities in a subset of patients in whom both muscles were analysed.Results103 ALS patients were included in the study. Neurophysiological genioglossus involvement was associated with a shorter survival (p = 0.002), a shorter time to moderate dysphagia (p = 0.0001) and to severe dysarthria (p = 0.012). Its prognostic value was still evident in patients without clinical bulbar LMN signs. Bulbar clinical LMN signs were only associated with an earlier onset of moderate dysphagia (p = 0.0001). EMG masseter abnormalities did not reach statistical significance with regard to all the clinical milestones.ConclusionsGenioglossus EMG at diagnosis could provide important information about ALS progression rate. The masseter muscle seems to be less involved in ALS.SignificanceEMG genioglossus involvement is a prognostic factor in ALS.     

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     

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.     

Distal Predominance of Electrodiagnostic Abnormalities in Early‐Stage Amyotrophic Lateral Sclerosis

Introduction: In this study we compared the electrodiagnostic (EDX) yield of limb muscles in revealing lower motor neuron (LMN) dysfunction by electromyography (EMG) in early‐stage amyotrophic lateral sclerosis (ALS). Methods: This investigation was undertaken as a retrospective review at a single center. Results: Our study included 122 consecutive patients with possible ALS, as defined by revised El Escorial criteria. Distal limb muscles showed more frequent EMG abnormalities than proximal muscles. EDX yield was found to be higher in the limb where weakness began and when clinical signs of LMN dysfunction were evident. Adoption of the Awaji criteria significantly increased the yield of EMG‐positive segments in the cervical (P < 0.0005) and lumbosacral (P < 0.0001) regions, and upgraded 19 patients into the probable category and 1 patient into the definite category. Discussion: EMG abnormalities are predominant in the distal limb in early‐stage ALS. A redefinition of an EDX‐positive cervical or lumbosacral segment, with an emphasis on distal limb muscles, may result in an earlier ALS diagnosis. Muscle Nerve 58 : 389–395, 2018     

Assessment of thoracic paraspinal muscles in the diagnosis of ALS

The distribution of muscle involvement, assessed clinically and electromyographically, was analyzed prospectively in 55 consecutive amyotrophic lateral sclerosis (ALS) patients and in 54 patients with other predominantly motor syndromes, some of whom were referred with suspected ALS. In ALS patients, distal limb muscles and thoracic paraspinal muscles were affected most frequently, more so than proximal limb and cranial muscles. The incidence of bulbar symptoms in ALS was greater in women than in men. These patterns suggest selective vulnerability of specific neuronal populations. The vulnerability of truncal muscles, illustrated by thoracic paraspinal wasting or head and shoulder drooping, was a helpful differential sign in diagnosing ALS. Thoracic paraspinal electromyography was especially valuable in distinguishing ALS from other disorders, such as combined cervical and lumbar spondylotic amyotrophy or polymyositis, which may masquerade as ALS. The finding of denervation atrophy on biopsy of thoracic paraspinal muscles was diagnostic in difficult cases. Because the thoracic paraspinal muscles are frequently affected in ALS and spared in spondylotic amyotrophy, their assessment provides a practical strategy in differentiating ALS from other motor syndromes.     

Clinically definite ALS presenting weeks after mild electric injury: causality or coincidence?

Motor neuron syndromes including typical ALS develop very rarely after electrotrauma, with possible causality discussed but not confirmed. We report on a 44-year-old male who developed clinically definite ALS by the revised El Escorial criteria with onset weeks after mild electric injury. He presented with asymmetric upper limb amyotrophy and weakness beginning around the entry point of the current. Over 1?year he developed generalized wasting, weakness and fasciculations, including the bulbar and thoracic muscles, with prominent spasticity and pyramidal tract signs. Electrodiagnostic studies confirmed widespread denervation, very unstable neurogenic motor units in the bulbar, cervical, thoracic and lumbosacral segments with normal motor velocities and normal sensory parameters. This is a well-documented case of fast-progressive ALS that seems related to electric injury.     

AAEM case report #5: Amyotrophic lateral sclerosis

A 40-year-old man presented with a gradual onset of gait unsteadiness and weakness in the arms. The stretch reflexes were normal in the upper extremities but hyperactive in the lower extremities with bilateral Babinski signs. A myelogram revealed a partial obstruction at C-5–6. Two prior electromyograms, 7 and 5 months prior to admission, reportedly showed positive waves only in two peroneal supplied muscles. Repeat electromyographic testing demonstrated normal nerve conduction velocities and needle electrode abnormalities in upper and lower extremities as well as thoracic paraspinal muscles allowing a diagnosis of amyotrophic lateral sclerosis (ALS). The importance of electromyographic testing in clinically nonaffected areas is stressed as well as its role in patients presenting with upper motor neuron signs. It is the task of the clinical electromyographer to consider other entities in the differential diagnosis, such as a multifocal motor neuropathy with conduction blocks and design the tests accordingly. The role of electromyography in the prediction of the course of ALS by assessing the degree of reinnervation is discussed. This will become increasingly important in the design of treatment trials.     

Is magnetic resonance imaging a plausible biomarker for upper motor neuron degeneration in amyotrophic lateral sclerosis/primary lateral sclerosis or merely a useful paraclinical tool to exclude mimic syndromes? A critical review of imaging applicability in clinical routine

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that affects motor neurons in the cerebral cortex, brainstem, and spinal cord, brain regions in which conventional magnetic resonance imaging is often uninformative. Although the mean time from symptom onset to diagnosis is estimated to be about one year, the current criteria only prescribe magnetic resonance imaging to exclude "ALS mimic syndromes". Extensive application of non-conventional magnetic resonance imaging (MRI) to the study of ALS has improved our understanding of the in vivo pathological mechanisms involved in the disease. These modern imaging techniques have recently been added to the list of potential ALS biomarkers to aid in both diagnosis and monitoring of disease progression. This article provides a comprehensive review of the clinical applicability of the neuroimaging progress that has been made over the past two decades towards establishing suitable diagnostic tools for upper motor neuron (UMN) degeneration in ALS.