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     

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。     

运动神经元疾病合并脊髓型颈椎病

目的：探讨运动神经元疾病合并脊髓型颈椎病的临床表现、诊断和治疗。方法：对合并两病的１２例患者进行临床分析并结合文献讨论。结果：两病并存者,年龄偏大,以波及高位颈髓居多,胸锁乳突肌肌电图和上肢皮节体感诱发电位有助鉴别。结论：运动神经元疾病和脊髓型颈椎病可以同时存在,脊髓型颈椎病手术治疗应视运动神经元疾病病情而定。     

运动神经元病患者的肛门外括约肌肌电图特征

目的总结运动神经元病(motor neuron disease,MND)患者的肛门外括约肌肌电图(EAS-EMG)特点。方法分析20例不同临床亚型MND患者的EAS-EMG资料,并与无二便障碍症状和体征的阴性对照组(对照组)对比。结果与对照组〔(12.21±2.26)ms、(512.89±363.55)(ms.μV)〕比较,MND患者的肛门外括约肌(EAS)运动单位电位(motor unit potential,MUP)平均时限延长〔(14.55±2.27)ms,P=0.000〕,面积增大〔(718.47±236.57)(ms.μV),P=0.05〕,MUP波幅、多相波比例、平均相在两组间无统计学差异(P>0.05)。结论 MND患者EAS存在临床下神经源性损害,MUP时限、面积是反映MUP特征的敏感指标。     

单纤维肌电图在68例运动神经元病患者中的研究

目的　研究运动神经元病 (MND)患者的单纤维肌电图 (SFEMG)改变、病理生理机制及临床意义。方法　对 6 8例 (男 4 3例 ,女 2 5例 )经病史、临床表现和神经电生理检查测定证实的MND患者进行了伸指总肌SFEMG测定 ,并与年龄匹配的正常人对照。结果　所有患者感觉神经传导速度测定均正常 ,肌电图为广泛神经源性损害。SFEMG检查发现 :颤抖值 (jitter)为 30～ 178μs,平均 (80 2± 32 6 ) μs ;jitter>5 5 μs占 5 0 %～ 10 0 0 % ,平均 6 0 6 %± 2 9 0 % ;阻滞 (block)所占百分比为 0 0 %～90 0 % ,平均 2 9 3%± 30 0 % ;纤维密度 (FD)为 1 4～ 4 0 ,平均 2 6± 0 6。其中 5 1例确诊和拟诊的肌萎缩侧索硬化患者jitter增宽、block和FD增高最明显。还发现伸指总肌肌无力的程度与jitter增宽和block百分比呈明显的负相关。结论　SFEMG的异常改变在确诊患者组最明显 ;jitter增宽、block百分比升高和FD增高与肌肉无力的严重程度呈明显的负相关 (P <0 0 0 1) ;jitter增宽、block百分比升高和FD增高反映进行性失神经、神经再生和神经肌肉接头处的传递情况     

159例运动神经元病患者的临床和肌电图特征分析

目的 分析运动神经元病(MND)患者的临床特征以及神经肌电图的特征性表现.方法 选择2015年3月至2021年7月河南科技大学第一附属医院神经内科收治的159例MND患者,收集其临床和神经电生理资料.结果 MND患者运动神经受损而感觉神经传导正常(年龄>50岁和糖尿病患者除外).159例患者针极肌电图发现颅、颈、胸、腰...     

运动神经元病的单纤维肌电图的研究

目的研究单纤维肌电图（ＳＦＥＭＧ）对运动神经元病的辅助诊断价值及探讨单纤维肌电图异常率与其病型、病情的关系。方法应用单纤维肌电图技术对４８例运动神经元病（ＭＮＤ）患者，２４例颈椎性脊髓病（ＣＳＭ）患者及４２名健康正常受试者进行研究。结果ＭＮＤ患者的ＳＦＥＭＧ阳性率为７３％，ＣＳＭ患者的ＳＦＥＭＧ阳性率为４％。结论ＳＦＥＭＧ有助于鉴别ＭＮＤ与ＣＳＭ。ＳＦＥＭＧ异常率与病型无关，与病情有关，提示ＭＮＤ存在明显的神经肌肉接头传递功能障碍。     

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。     

运动神经元疾病患者的临床肌电图特征

目的探讨神经电生理肌电图检测在运动神经元疾病患者诊断中的价值。方法对20例确诊为运动神经元疾病的患者进行神经传导速度和针极肌电图检测与分析。结果广泛的神经源性损害普遍存在于运动神经元患者,部分患者发现插入电位延长,不同程度自发电位,其中纤颤电位15例(75.0%),正锐波13例(65.0%),束颤电位9例(45.0%),所有患者出现运动单位电位时限延长、波幅增高。5例(25.0%)运动神经传导速度显示轻度下降。结论神经电生理肌电图为运动神经元疾病的诊断及鉴别诊断提供了客观可靠的依据,对早期发现亚临床病变也有一定的意义。     

Dementia with motor neuron disease

Dementia with motor neuron disease has been described as a new clinicopathologic entity and more than 100 cases have been reported in Japan since 1964. The clinicopathologic criteria in the diagnosis of dementia with motor neuron disease are: (i) frontotemporal lobe‐type dementia with insidious onset, mostly in the presenile period; (ii) neurogenic muscular wasting during the course of the illness (amyotrophic lateral sclerosis‐ or SPMA‐like symptoms); (iii) duration from the onset of illness to death of 2–5 years (average, 30.6 months); (iv) both extrapyramidal symptoms and definite sensory deficits are present less commonly; (v) no characteristic abnormalities in the cerebrospinal fluid or electroencephalogram on screening; (vi) no known parental consanguinity or familial occurrence; and (vii) non‐specific, mild to slight degenerative changes in the frontotemporal cortex, hypoglossal nuclei and spinal cord, and frequently in the substantia nigra. Dementia with motor neuron disease is characterized by ubiquitin‐immunoreactive intraneuronal inclusions in cortical layer II and hypocampal dentate granule cells.     

The reorganization of motor units in motor neuron disease

We studied 78 patients with motor neuron disease (MND) using concentric needle electromyography. Analysis on weak and maximal effort was performed using our own, fully automated, computer method, EMG-LAB. In addition to the conventional parameters of single motor unit action potentials (MUAPs) and interference pattern, new criteria were applied: the range of the acting motor units and the functional recruitment order. A total of 375 muscles of MND patients and 120 control muscles were investigated. The electromyographic data were analyzed separately in five groups of muscles, classified A, B, C, D, and E according to their clinical condition. Those results allowed us to discern six neurophysiological stages (N _{0, 1, 2, 3, 4, 5}) from the early to the most advanced phase. It has been confirmed that reinnervation in MND is adequate to compensate for the loss of over 50% of motor neurons but it is only a transitory phase in the morbid course. At stages N_0–5, the electrophysiological data reflect structural and functional integrity of the functioning motor units. Evaluation of not only single MUAPs but also of the full range of acting motor units and their recruitment order allowed a deeper look into the underlying pathophysiological mechanisms. © 1997 John Wiley & Sons, Inc. Muscle Nerve, 20, 306–315, 1997.     

Cognitive impairment in motor neuron disease

A systematic investigation of the cognitive functions of 22 patients affected with motor neuron disease (MND) compared to 36 controls matched for age and education was performed. The MND group showed cognitive performances slightly but significantly lower than the control group; 6 MND patients, however, had decidedly pathological values. Cognitive impairment was stereotyped and global, with sparing of memory. There was no significant difference between patients with isolated involvement of the lower motor neuron and those with associated pyramidal involvement. Our neuropsychological findings are in agreement with previous clinical, neuroradiological and pathological reports indicating extra-motor cerebral involvement in MND.     

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.     

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.     

Diagnostic investigation and multidisciplinary management in motor neuron disease

Abstract Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease. ALS is a progressive neurodegenerative disorder, involving motor neurons in the cerebral cortex, brainstem and spinal cord, presenting with a combination of upper and lower motor neuron signs. Etiology remains undetermined, although a multifactorial origin is widely accepted including genetic factors, auto-immunity, oxidative stress, glutamate excitotoxicity and abnormal neurofilament aggregation. The absence of specific diagnostic testing, and variable clinical presentations make the diagnosis of ALS challenging, relying upon correlation of clinical, electrophysiological and neuroimaging data. The disease is relentlessly progressive, with dysarthria, dysphagia, tetraparesis, and respiratory insufficiency due to ongoing respiratory muscle paresis. There is no specific treatment for ALS. Riluzole, a glutamate antagonist, is the only FDA approved drug for ALS, but has only a modest effect on survival. The multiplicity and progressiveness of the disabilities in ALS, highlights the need for a coordinated multidisciplinary rehabilitation program managing symptoms, respiratory care, dysphagia and nutrition, dysarthria and communication, physical and occupational therapy. The main goals are to prolong independence, prevent complications and improve quality of life.     

Central motor conduction in cerebrovascular disease and motor neuron disease

Conduction in the central motor pathways was studied in 9 patients with cerebrovascular disease (CVD), 13 with amyotrophic lateral sclerosis (ALS) and 3 with spinal progressive muscular atrophy (SPMA). Motor responses evoked in the limb by cortical, cervical and lumbar stimulations were recorded. The central conduction time (CCT) was calculated for each muscle. In patients with CVD, responses to cortical stimulation were unobtainable or delayed in the paretic limb muscles. In patients with ALS the abnormality of central motor conduction had significant correlation with the extensor plantar response. The CCTs were normal in patients with SPMA. This technique demonstrated a subclinical lesion in some patients. We conclude that the new technique of examining motor conduction along the corticospinal tract may be useful to detect a subclinical lesion in the corticospinal tract.     

Cyanide metabolism in motor neuron disease

Cyanide concentrations in whole blood, saliva and urine were measured in 83 patients with motor neuron disease (MND) and age-, sex-matched control subjects consisting of 62 patients with and 49 without neurological disorders. Cyanide levels in whole blood and urine of MND patients were significantly higher than the non-neurological control groups in smokers and non-smokers. Cyanide levels in whole blood of MND patients were also higher than the neurologic control group in smokers, but not in non-smokers. There was no significant difference between the cyanide level and either the clinical types or degree of disability of MND. The results suggest that MND patients possess a disorder in cyanide metabolism.