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±     

经颅磁治疗对延髓背外侧综合征患者吞咽构音功能及生存质量的影响

目的研究对延髓背外侧(Wallenberg)综合征伴吞咽困难患者给予颅磁治疗后患者吞咽、构音功能及生存质量变化。方法选取选取2017年2月～2019年7月我院收治Wallenberg综合征患者,随机随机分为观察组(n=55)与对照组(n=55)。观察组患者给予常规药物治疗及颅磁治疗,对照组患者给予常规药物治疗及康复训练。采用洼田饮水试验评价两组患者治疗前后吞咽功能,采用吞咽造影技术测定治疗前后吞咽时舌骨位移大小。通过吞咽喉镜检查,采用Rosenbek误吸程度分级评估治疗前后渗漏误吸程度。采用言语功能评定法评价两组患者治疗前后构音功能,采用世界卫生组织生存质量测定简表(WHOQOL)评估两组患者治疗前后生存质量。结果治疗后观察组患者吞咽功能异常率32.73(18/55)明显低于本组治疗前100%(55/55)及对照组56.36%(31/55)(P<0.05);治疗后对照组吞咽功能异常率56.36%(31/55)低于本组治疗前100%(55/55),(P<0.05)。治疗后两组患者进食3种不同食物的舌骨最大位移均大于本组治疗前(P<0.05);治疗后观察组患者进食3种不同食物的舌骨最大位移均大于对照组(P<0.05)。治疗后观察组Rosenbek分级2级、3级、4级比例明显高于本组治疗前,6级、7级、8级比例明显低于本组治疗前(P<0.05);治疗后观察组2级、3级、4级、5级比例高于对照组,6级、7级、8级比例低于对照组(P<0.05)。治疗后观察组患者治疗有效率96.36%(53/55)明显高于对照组78.18%(43/55)(P<0.05)。治疗后观察组患者构音功能障碍率41.82%(23/55)显著低于本组治疗前89.09%(49/55)及对照组70.91%(39/55)(P<0.05);治疗后对照组患者构音功能障碍率70.91%(39/55)低于本组治疗前87.27%(48/55)(P<0.05)。治疗后观察组构音功能障碍治疗有效率72.73%(40/55)高于对照组61.82%(34/55)(P<0.05)。治疗后两组患者WHOQOL量表各维度得分及总分均高于本组治疗前及对照组(P<0.05)。结论颅磁治疗可有效改善Wallenberg综合征伴吞咽困难患者吞咽功能及构音功能障碍情况,明显提高治疗后患者生存质量。颅磁刺激治疗法可于临床广泛使用。     

多发性肌炎患者神经电生理特点分析

目的增进对多发性肌炎患者肌电图特点的认识,提高其检查的阳性率。方法对91例多发性肌炎患者进行肌电图(EMG)、神经传导速度测定。结果肌电图异常率为87.9%,肌源性损害者占79.1%,神经源性损害者占8.8%。其中插入电位延长、自发电位的阳性率分别为6%和52%,肱二头肌出现率较高,外展拇短肌出现率最低(p<0.05);运动单位电位(MUP)时限缩短的阳性率为71%,胫前肌出现率最高,外展拇短肌最低(p<0.05);MUP波幅降低的阳性率较低,仅为7%;多相波增多的阳性率为29%,胫前肌出现率最高,股四头肌最低(p<0.05);重收缩时波形异常的阳性率为26%,以股四头肌出现率最高;重收缩时峰值电压降低的阳性率为31%,胫前肌出现率最高,外展拇短肌最低(均p<0.05)。5例患者EMG呈神经源性损害,1例感觉神经传导速度减慢,2例运动神经传导速度减慢。肌电图正常组、肌源性损害组及神经源性损害组患者的病程、年龄无明显差异。结论 EMG对多发性肌炎诊断的阳性率为87.9%,其中以MUP时限缩短出现率最高为71%,其次为自发电位为52%。EMG异常最多见于肱二头肌、股四头肌和胫前肌。     

肌活检在肌病诊断准确性的临床研究-单中心1年期间56例患者的回顾性分析

目的 探讨肌肉活体组织检查(肌活检)的病理表现并结合临床、电生理、肌肉影像学及相关实验室检查在肌肉疾病中的诊断价值。方法 收集2017年7月-2018年7月在本院行肌活检的56例患者,分析肌肉病理学与人口学、肌电图、肌肉磁共振、血清肌酸激酶、相关抗体和基因检测的相关性,并评估其诊断价值。结果 ①男36例,女20例,年龄5～69岁、平均年龄(39.2±18.6)岁; ②肌电图显示肌源性损害者占83.6%(46/55),其中6例合并强直电位,6例合并神经源损害; 可疑肌源性损害12.7%(7/55); 单纯神经源性损害1.8%(1/55); 正常1.8%(1/55); ③肌肉磁共振异常率87.9%(29/33); 肌酸激酶升高者60%(33/55),中位823[349, 2505.5] U/L; 4例进行肌炎相关抗体检查者3例异常; 17例进行基因检查者12例提示肌病诊断,其中仅6例家族史阳性; ④通过肌肉组织病理检查并结合临床、抗体和基因检测80.4%的患者(45/56)能够明确肌病诊断,明显高于肌活检之前(23.2%)(P<0.01)。结论 肌活检能明显提高肌病的准确诊断率。     

Hhcy对缺血性脑卒中患者再发缺血性血管事件及相关死亡事件的影响

目的探讨高同型半胱氨酸血症(hyperhom ocysteinemia,Hhcy)对缺血性卒中(IS)患者再发、死亡及冠状动脉和外周动脉缺血性事件发生的影响,为进一步对缺血性血管病的综合干预提供依据。方法检测245例IS患者空腹血浆同型半胱氨酸(Hcy)水平,并参照同期选取的52例健康体检者为对照者按血浆Hcy水平将其分为Hhcy组和非Hhcy组,追踪观察两组患者IS再发、死亡及冠状动脉和外周动脉缺血事件的发生情况。结果其中Hhcy组IS再发率(52.63%)显著高于非Hhcy组(24.67%)(P<0.01);死亡事件发生率(12.63%)亦显著高于非Hhcy组(3.33%)(P<0.01);冠脉缺血事件发生率(18.95%)明显高于非Hhcy组(10.00%)(P<0.05);外周动脉缺血事件发生率两组分别为5.26%和2.67%,差异无统计学意义(P>0.05);并发2种以上缺血性事件的患者Hhcy组(17.89%)显著多于非Hhcy组(5.33%)(P<0.01);Hhcy组患者5年内缺血性血管事件发生率(57.89%)显著高于非Hhcy组(32.00%)(P<0.01);Logistic回归分析发现,Hcy升高是IS患者缺血性血管事件再发的独立危险因素(OR分别为1.174;95%CI1.119~1.233;P<0.05)。结论 Hhcy与IS患者缺血性血管事件再发及死亡预后密切相关。     

单纤维肌电图对糖尿病周围神经病变的应用价值

目的探讨单纤维肌电图(SFEMG)对糖尿病周围神经病变(DPN)的应用价值。方法应用SFEMG检测129例DPN患者的优势侧指总伸肌颤抖(jitter)和纤维密度(FD),按常规方法行神经传导速度(NCS)检测。比较SFEMG和NCS的异常检出率,并分析jitter值与血糖化血红蛋白(HbA1C)和预后的关系。结果 SFEMG异常检出率(91.5%)显著高于NCS(78.3%)(P<0.01)。HbA1C轻度升高组SFEMG异常检出率(86.4%)显著高于NCS异常检出率(69.7%)(χ2=7.69,P<0.01),而HbA1C重度升高组差异无统计学意义。jitter值与HbA1C水平呈正相关(r=0.3132,P<0.05)。jitter值正常及轻度异常患者治疗有效率(82.3%)及治愈率(30.6%)均显著高于明显异常者(54.2%,11.9%)(χ2=11.02,P<0.01;χ2=6.32,P<0.05)。结论 SFEMG对DPN的诊断意义显著,并且可用于DPN的预后判断。     

神经传导速度检测诊断酒精性周围神经病的价值

目的探讨神经传导速度(NCV)对慢性酒精中毒性周围神经病(CAPN)的诊断价值。方法采用肌电图检测52例CAPN患者的正中神经、尺神经、腓神经和胫神经的NCV,并与26例健康者进行对照比较。结果CAPN患者的NCV异常率为73.12%,明显高于对照组;下肢NCV异常率(80.77%)高于上肢异常率(76.47%);感觉神经传导速度(SCV)异常率(82.73%)高于运动神经传导速度(MCV)异常率(75.26%)。结论NCV检测可作为酒精中毒性周围神经病的方法之一。     

42例强直性肌营养不良症(MYD)电生理表现

为了研究探讨MYD患者的电生理特点,本文对42例MYD病人进行了肌电图(EMG)、神经传导速度(NCV)和体感诱发电位(SEPs)检查。结果发现,全部患者均有电肌强直表现,其放电频率以拇短展肌最高。28%的病人发现NCV减慢,以运动神经为主,且早年症状组NCV异常率明显高于成年症状组病人。SEPs异常检出率为24%,且多数合并周围神经损害。结论:42例MYD患者存在着广泛的电生理异常,且早年症状组较成年症状组病人更为常见。     

运动神经元病患者血清和脑脊液中NSE和GM1抗体测定及其临床意义

目的　通过测定运动神经元病(MND)患者血清和脑脊液(CSF)中神经元特异性烯醇化酶(NSE)和GM1抗体,初步探讨其临床意义。方法　应用ELISA法测定30例MND患者血清和CSF中NSE和GM1抗体水平。结果　MND组CSF和血清中NSE含量均明显高于NC组(P<0.01,P<0.05),而仅在CSF中NSE含量高于OND组(P<0.01);临床分型以运动神经元损害为主的进行性脊肌萎缩症(PSMA)患者CSF中NSE含量明显高于其他组(P<0.01),而血清中NSE水平在各临床类型间差异并不明显(P>0.05);MND患者血清和CSF中不论是IgM型或是IgG型GM1抗体阳性率均明显高于OND组和NC组(P均<0.01);MND患者血清和CSF配对样品NSE水平有较好的相关性(r=0.502,P<0.01),MND患者CSF中NSE水平与GM1抗体亦有相关性(r=0.475,P<0.05),其余各组之间均未见明显相关性(P>0.05)。结论　MND患者CSF中NSE和GM1抗体水平可以反映运动系统神经元损害的程度和范围;推测这种神经元的损伤可能与GM1抗体介导的中枢神经系统免疫反应有关。     

进行性脊肌萎缩症129例临床分析

目的探讨进行性脊肌萎缩症(PSMA)的临床特点、诊断与鉴别诊断。方法回顾性分析129例PSMA患者的临床资料。结果本组患者均隐袭起病,逐渐加重,男性多见,发病年龄65.9%患者>50岁。首发症状以单侧上肢无力和肌萎缩为多见(65.9%),均表现为下运动神经元损害的症状和体征,51.9%患者出现延髓麻痹症状;肌电图检查均提示神经源性损害;易误诊为颈或腰椎病。结论本病是一组慢性进行性下运动神经元疾病,病变可累及延髓。诊断主要依据临床表现和肌电图。     

Dynamic change of proximal conduction in demyelinating neuropathies: a cervical magnetic stimulation combined with maximum voluntary contraction.

OBJECTIVE: To evaluate conduction abnormalities in the nerves innervating the proximal muscles in demyelinating neuropathies (DN) using cervical magnetic stimulation. METHODS: We applied cervical root magnetic stimulation in the biceps brachii muscles and examined its activity-dependent conduction changes produced by maximal voluntary contraction (MVC) in 12 DN patients (seven chronic inflammatory demyelinating polyradiculoneuropathy and five multifocal motor neuropathy), six motor neuron disease (MND) patients, and 12 healthy volunteers. RESULTS: Defining the upper normal limit of motor threshold (31%) and latency (6.7 ms) of the compound muscle action potential (CMAP) as mean+2SD, most DN patients revealed an abnormality in motor threshold (10/12) and latency (11/12) in contrast to MND patients (motor threshold (1/6) and latency (0/6)). These parameters contribute to the differentiation of DN from MND (P<0.01). Furthermore, the MVC maneuver transiently decreased the CMAP amplitude ratio (after MVC/before MVC x 100) in DN (83+/-18 %) compared with MND (P<0.01). Two of three DN patients who showed normal motor threshold or latency as in MND were successfully differentiated from MND by the MVC maneuver. CONCLUSIONS: In DN patients, conduction abnormality in the nerves innervating the proximal muscles was revealed by cervical magnetic stimulation combined with the MVC maneuver. SIGNIFICANCE: Our results suggested that conduction abnormalities in the proximal nerves innervating the proximal muscles could be evaluated by this method.     

Motor neuron disease: usefulness of transcranial magnetic stimulation in improving the diagnosis.

Clinical upper motor neuron (UMN) involvement is sometimes difficult to detect in motor neuron disease (MND). For this reason we performed transcranial magnetic stimulation (TMS) to find out whether this technique may be useful in revealing signs of pyramidal tract impairment. Fifty-five MND patients, clinically divided into 22 amyotrophic lateral sclerosis (ALS), 18 ALS with probable UMN signs (ALS-PUMNS), 10 pure lower motor neuron syndrome (LMNS), and 5 progressive bulbar palsy (PBP), underwent standard TMS, recording from abductor digiti minimi and flexor allucis muscles. Prolongation of cortical motor evoked potential (MEP) latency and central conduction time (CCT) and absent MEP were considered as pathologic. ALS-PUMNS and LMNS patients were clinically reclassified after 1 year. TMS was abnormal in 95.4% of ALS, 72.2% of ALS-PUMNS, 50% of LMNS and 20% of PBP. Correlations between TMS parameters and both clinical signs of UMN involvement and disease severity were highly significant. TMS showed a high sensitivity, but lacked specificity. After 1 year, 11 patients among the ALS-PUMNS group were clinically reclassified as definite ALS: all of them had shown TMS abnormalities at the first examination. In conclusion, TMS provides important diagnostic information for an early prediction of ALS in those MND patients presenting with clinically equivocal UMN impairment.     

Coughing and choking in motor neuron disease

OBJECTIVES: To assess the frequency and severity of coughing and choking episodes, possible related factors, and their association with chest infections in patients with motor neuron disease (MND). METHODS: Thirty seven patients with MND and 23 healthy volunteers were studied. Cough was assessed using a questionnaire and a 3 day diary, and volitional cough quantified by peak cough flow and sound intensity. Other clinical symptoms, smoking habit, affective state, oral secretions, bulbar signs, and quantitative assessments of swallowing and respiratory function were documented. RESULTS: Patients with MND coughed and choked significantly more often and to a greater degree than the healthy volunteers (26 of 37 patients with MND and 2 of 23 volunteers, p<0.001). Female sex, older age, abnormal speech, reduced swallowing capacity, and low forced vital capacity (FVC)% predicted were each significantly associated with excessive coughing and choking episodes in patients with MND. Smokers had significantly more severe and prolonged episodes of coughing and choking than non-smokers (p<0.05). Patients with upper motor neuron bulbar signs had a greater tendency to severe and prolonged episodes of coughing and choking than those without (p<0. 05). Chest infections were reported only rarely among the patients who coughed and choked. CONCLUSIONS: Coughing and choking episodes are common in patients with MND but infrequently associated with overt chest infection. Upper motor neuron bulbar signs may both promote factors (for instance, dysphagia) which trigger cough and reduce volitional capacity to suppress it.     

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     

98例神经肌肉病的临床、肌电图与病理研究

目的 探讨肌电图（EMG），肌活检对神经肌肉病的诊断价值。比较EMG，肌活检及初始临床诊断3者之间的关系。方法 将98例神经肌肉病分成肌病。重症肌无力和运动神经元病3组进行研究。结果 肌病组（80例），68.8%(55/80)肌活检，75%（60/80）EMG呈肌源性损害；重症肌无力组（10例）；针极EMG（不包括重视频率电刺激）及肌活检均未显示特异性改变；运动神经元病组（8例），75?/8）肌活检，100%（8/8）EMG呈神经源性损害。结论 肌活检对肌病明确诊断可提供直接信息。对运动神经元病只能做出神经源性损害结果。缺乏特异性。EMG对神经肌肉病只能做出分类诊断；单纯凭借初始临床资料易导致该类疾病误诊。     

Clinical and epidemiological features of motor neuron disease in south-western Greece

OBJECTIVE: To evaluate the epidemiological and clinical features of motor neuron disease (MND) in a region (835,000 inhabitants) of south-western Greece. PATIENTS AND METHODS: The medical records of all patients diagnosed with adult-onset MND at the Department of Neurology of the University Hospital of Patras from 1990 to 2003 were reviewed. RESULTS: Overall 133 patients were identified, corresponding to a mean annual incidence rate of 1.13/100,000 population with male preponderance. Eighty-five of them were males (63.9%) and 48 (36.1%) females with a mean age of 61.4 +/- 13.3 years. The most common type of MND was amyotrophic lateral sclerosis (ALS) being identified in 111 (83.5%) patients, whereas 19 cases (14.3%) were classified as progressive spinal muscular atrophy (PSMA) and three (2.2%) cases as progressive bulbar palsy (PBP). The mean age at onset was 60.3 +/- 13.5 years, while the mean delay between age at onset and age at diagnosis was 1.3 +/- 1.1 years. The symptoms at onset involved the lower limbs in 76 (57.2%) cases, upper limbs in 32 (24%) cases, bulbar region in 22 (16.5%) cases and respiratory muscles in three (2.3%) cases. The mean survival time after onset of disease was 20.4 +/- 8.3 months for ALS patients, 15.3 +/- 4.5 months for PBP and 38.1 +/- 26.4 months for PSMA patients. CONCLUSIONS: There was no statistically significant difference in the results of the considered epidemiological parameters of our study to those reported by other similar studies. The study of the patients with MND showed a predominance of ALS patients. No potentially causative clinical associations were found and no relation between socioeconomic factors, occupational exposure and the disease was noted.     

Somatosensory evoked potentials in motor neuron disease

We studied median somatosensory evoked potentials (SEPs) in an unselected series of 30 patients with sporadic motor neuron disease (MND). SEPs were affected in 17 patients (57%), with a higher incidence of abnormality in amyotrophic lateral sclerosis and bulbar palsy than in progressive muscular atrophy. In a majority of patients, simultaneous bilateral stimulation of the median nerve revealed a delay or absence of scalp-recorded central N32 and/or N60, leaving the earlier peaks intact. In the remaining cases, the N19 peak was asymmetrically prolonged. These findings suggest common involvement of somatosensory pathways in MND, either at cortical or subcortical levels, and correlate with neuropathologic reports of neuronal degeneration beyond the primary motor system.     

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.     

急性脑梗死患者临床分型及梗死面积与心电图改变的关系

目的 探讨急性脑梗死患者临床分型和梗死面积与心电图(ECG)改变的关系.方法 给216例急性脑梗死患者进行ECG检查,按牛津郡社区卒中项目(OCSP)分型和梗死面积分型,对各组患者的ECG检查结果进行分析比较.结果 OCSP分型完全前循环梗死(TACI)组、部分前循环梗死(PACI)组、后循环梗死(POCI)组和腔隙性梗死(LACI)组患者的ECG异常率分别是: 95. 5%、80.4%、62.5%和48.5%,TACI组和PACI组明显高于LACI组(P＜0.05～0.01);大中面积梗死组(83.7%)的ECG异常率明显高于小面积梗死组(60.4%)和腔隙性梗死组(53.2%)(P＜0.05～0.01);小面积梗死组的ECG异常率高于腔隙性梗死组(P＜0.05).OCSP和梗死面积分型各亚型组出现ST-T改变和心律失常的比率差异有统计学意义(P＜0.05～0.01).结论 急性脑梗死临床分型病情重和梗死面积大的患者ECG异常率高.