三重经颅磁刺激技术对肌萎缩侧索硬化上运动神经元损害的评价及其作用

目的 研究三重经颅磁刺激技术(triple stimulation technique,TST)在肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)患者上运动神经元(upper motor neuron,UMN)损害的评价和诊断中的作用.方法 收集我院门诊和病房收治的ALS患者50例和健康志愿者22名进行右上肢小指展肌TST测定、中枢运动传导时间(central motor conduction time,CMCT)测定、运动诱发电位(motor evoked potential,MEP)潜伏期、静息运动阈值(resting motor threshold,RMT)、复合肌肉动作电位(compound muscle action potential,CMAP)测定及对TST与改良的Ashworth评分(Modified Ashworth Scale,MAS)、医学研究委员会评分、修订的ALS功能评分、病情进展速度等进行相关性分析.结果 根据EI Escorial的ALS诊断标准,临床确诊5例,临床拟诊19例,实验室辅助拟诊24例,临床可能2例.ALS患者右上肢有UMN体征组[28例,62.0%(40.7%,75.9%)]与无UMN体征组[22例,95.6%(85.4%,100.0%)],健康对照组(96.9%±2.6%)比较,TST波幅比[M50(M25,M75)]差异均有统计学意义(Z=-4.827、-5.435,均P=0.000).有UMN体征组(89.3%)与无UMN体征组(27.3%)、健康对照组(9.1%)TST波幅比异常出现率差异均有统计学意义(χ2=20.109、31.897,均P=0.000),无UMN体征组与健康对照组差异无统计学意义(χ2=1.375,P=0.241).TST波幅比、MEP潜伏期、易化状态下MEP潜伏期、CMCT、RMT对ALS患者UMN体征的阳性检出率分别为89.3%、64.3%、53.6%、64.3%、78.6%.TST波幅比与右上肢腱反射(r=0.690)、MAS评分(r=-0.772)、诊断分级(r=0.483)存在相关性(均P=0.000),与RMT(r=-0.774,P=0.000)、MEP潜伏期(r=-0.444,P=0.005)、MEP/CMAPerb(r=0.685,P=0.000)、易化状态下MEP/CMAPerb(r=0.770,P=0.000)存在相关性.结论 TST为ALS患者UMN损害提供了敏感的检测指标,能够发现临床下UMN受累,较传统经颅磁刺激方法 提高了诊断的敏感性,能够对UMN损害进行半定量评价,提示其有可能成为监测病情变化的客观评价指标.     

三重磁刺激技术对上运动神经元损伤的评价作用

目的研究三重磁刺激技术(TST)的特点,探讨TST在评价运动神经元疾病患者上运动神经元(UMN)损害中的作用。方法选取肌萎缩侧索硬化(ALS)患者12例、下运动神经元综合征(LMNS)患者14例,分别进行双上肢小指展肌TST测定及运动诱发电位(MEP)潜伏期、中枢运动传导时间(CMCT)测定,并将TST波幅比与改良的医学研究委员会(MRC)评分、修订的肌萎缩侧索硬化功能评分量表(ALSFRS-R)评分、肺功能百分比、病程等进行相关性分析。结果 ALS患者12例均完成双侧TST检测,其尺神经TST波幅比为(55.8±32.6)%,较正常参考值下降(F=-3.448,P=0.002),TST波幅比阳性率为70.8%。LMNS患者共13例完成TST检测,其尺神经TST波幅比为(79.4±16.4)%,与正常参考值无统计学差异(F=0.215,P=0.832)。ALS组尺神经TST波幅比〔(55.8±32.6)%〕低于LMNS〔(79.4±16.4)%〕(F=-3.275,P=0.002)。TST波幅比评价UMN体征的敏感度76.5%,高于传统电生理指标;特异度为54.5%,低于传统电生理指标。TST波幅比与改良的MRC评分存在相关性(r=0.431,P=0.035),与ALSFRS-R评分、肺功能、病程、MEP潜伏期以及CMCT均无相关性。结论 TST可以发现亚临床UMN损害,其敏感性高于传统电生理观测指标。TST可以定量评估UMN受损程度,有可能用于ALS患者病情监测。     

咀嚼肌经颅电刺激运动诱发电位在肌萎缩侧索硬化诊断中的价值

目的　对肌萎缩侧索硬化 (ALS)患者进行经颅电刺激咀嚼肌诱发电位的研究 ,建立评估ALS头区皮质脑干束的检查方法。方法　对 2 0例ALS患者和 30名健康志愿者进行双侧咀嚼肌经颅电刺激 ,双侧同时接受 ,分别记录同侧的根运动诱发电位 (R MEP)和对侧的皮层运动诱发电位(C MEP)的潜伏期、波幅 ,计算中枢运动传导时间 (CMCT)。结果　R MEP可在所有ALS患者中引出。R MEP的潜伏期 [(3 4 4± 0 5 3)ms]、波幅 [(2 79± 2 19)mV]与健康对照组比较差异无显著意义 ;而C MEP有 12例ALS患者不正常 ,C MEP的潜伏期 [(7 6 9± 1 5 8)ms]与健康对照组 [(5 83±1 4 0 )ms]比较差异有显著意义 ,CMCT显著延长 (P <0 0 1)。结论　C MEP为一种非创伤性评估ALS头区上运动神经元受损的较敏感方法。     

弥散张量成像在肌萎缩侧索硬化中的应用

肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)的诊断主要依据El Escorial临床标准,结合电生理改变并除外其他疾病,其中下运动神经元(LMN)受累可通过肌电图诊断,而上运动神经元(UMN)损害主要依赖临床检查,缺乏客观评价标准.     

ALS功能评分和CMAP波幅对MND患者预后评估的研究

目的探讨MND患者ALSFRS与运动传导改变的相关性及其对预后评估的价值。方法2007年8月～2008年7月间符合Escorial诊断标准不同确定性水平的MND患者40例,健康志愿者102名为对照组。对所有患者均进行ALSFRS,并分别测量尺神经（腕部）-小指展肌以及胫神经（踝部）-[足母]展肌的复合肌肉动作电位（CMAP）波幅和末端运动潜伏期（DML）,分析ALSFRS与运动传导参数的关系。结果（1）肯定型ALS20例,拟诊型ALS10例,可能型ALS4例,进行性脊肌萎缩6例;（2）与对照组比较,患者组小指展肌和[足母]展肌CMAP波幅（mV）减低,尺神经DML延长（P〈0．01）;（3）ALSFRS与小指展肌和[足母]展肌CMAP波幅以及尺神经DML均呈显著相关（r分别为0．653,0．446和-0．592;P分别为0．000、0．004和0．000）;ALSFRS％30分的9例（100％）患者CMAP波幅均异常减低,31例ALSFRS≥30分的患者有17例（54．8％）的CMAP波幅异常减低（Х^2=6．25,P=0．012）;（4）随访的8例患者中6例ALSFRS在30分以下;与首次就诊相比,随访的8例患者其ALSFRS以及小指展肌CMAP波幅均减低（P〈0．05）。随访者的ALSFRS与小指展肌及[足母]展肌CMAP波幅均呈正相关（r分别为0．836和0．822;P分别为0．01和0．012）,与DML无相关（P〉0．05）。结论MND患者可出现CMAP波幅减低及DML延长;ALSFRS与CMAP波幅显著相关,二者同时减低提示预后差,可作为客观反映MND患者严重程度的可靠指标。     

急性脊髓炎患者磁刺激运动诱发电位的临床观察

目的探讨磁刺激运动诱发电位(MEP)技术对急性脊髓炎(AM)患者的辅助诊断价值。方法采用经颅磁刺激技术对30例急性脊髓炎患者及30例健康人分别于上肢的拇对掌肌及下肢的胫前肌进行MEP的检测。结果本组患者MEP均出现异常,其异常形式表现为锥体束传导时间(CMCT)阻滞、延迟或(和)波幅明显降低。结论 MEP技术可做为急性脊髓炎患者确诊的一种早期、快速、客观、简便的检查手段。     

肌萎缩侧索硬化患者下肢分裂现象的临床及电生理研究

目的探讨肌萎缩侧索硬化(ALS)患者的分裂足现象及其与电生理检测结果之间的关联。方法前瞻性收集2021年4月至2022年12月于解放军总医院第一医学中心神经内科住院的临床确诊和拟诊为ALS的患者, 收集同期因其他原因就诊于解放军总医院第一医学中心、电生理检查无异常者作为对照。通过采集ALS患者踝背伸肌和踝跖屈肌的肌力评分计算分裂腿[踝背伸肌修订版医学研究理事会肌力量表(mMRC)评分小于踝跖屈肌mMRC评分的肢体为分裂腿)的出现率, 采集ALS患者踇背伸肌和踇跖屈肌的肌力评分计算分裂足(踇背伸肌mMRC评分小于踇跖屈肌mMRC评分的肢体为分裂足)的出现率。检测全部受试者腓总神经和胫神经复合肌肉动作电位(CMAP)波幅, 对比ALS患者及对照者支配踝背伸肌和踝跖屈肌的运动神经元受累情况。使用受试者工作特征曲线(ROC)对腓总神经/胫神经 CMAP波幅比区分ALS患者和对照者的敏感度和特异度进行分析。结果最终共收集下肢受累(表现为下肢肌肉萎缩、无力或下肢mMRC评分小于13分)的ALS患者101例, 下肢肌力正常(无下肢肌肉萎缩、无力且下肢肌力mMRC评分等于13分)的对照者110名。在下...     

急性脑血管病患者经颅电刺激运动诱发电位及体感诱发电位的临床研究

目的 :研究经颅电刺激运动诱发电位 (MEP)和体感诱发电位 (SEP)与急性脑血管病 (ACVD)患者功能状态的关系。方法 :对 5 4例有偏瘫体征的ACVD患者行MEP和SEP检查 ,同时作肌力和临床神经功能评分测定。结果 :ACVD病人MEP的异常率为88 9%,主要表现为MEP缺失 ,潜伏期延长 ,波幅降低或波形异常 ,中枢运动传导时间 (CMCT)延长 ,患者与健侧及对照组比较 ,有显著差异 (P <0 0 1)。MEP缺失者 ,瘫痪重 ;MEP可引出者 ,瘫痪程度轻 ,两者间差异显著 (P <0 0 1)。SEP的异常率为 42 6 %,表现为中枢传导时间延长和皮质波异常或消失 ,功能评分为重型者 ,异常率高 ;轻型者 ,异常率低。结论 :MEP可定量分析ACVD病人运动功能的缺损情况 ,结合SEP可提供更多的脑部信息     

急性脑梗死运动诱发电位检测与临床相关性研究

目的 探讨急性脑梗死运动诱发电位变化特点与临床相关性.方法 94例患者均于入院当天行日常生活能力和临床神经功能缺损(MESSS)评分,并根据瘫痪程度进行分级,于入院3d内行MEP检查.结果 (1)病变部位不同,MEP的异常率不同.(2)MEP异常与病理征间无相关性.(3)MEP异常和MEP波形缺失与瘫痪程度、MESSS评分间具有正相关性,与BI评分具有负相火性,而皮质潜伏期和CMCT延长并不意味着患者病情严重.结论 MEP检查对判断脑梗死患者神经功能损伤严重程度具有重要意义.     

磁刺激运动诱发电位对脑梗死患者预后评估的价值

目的：研究运动诱发电位（MEP）对脑梗死患者预后的评价。方法：对105例首次发病的脑梗死患者在急性期行经颅磁刺激MEP检测。同时进行脑卒中临床神经功能损程度评价,1个月后再次进行评分及评定总的生活能力状态（病残程度评定）。结果：急性期MEP的异常率为68．5％,主要表现为皮层MEP消失、中枢运动传导时间（CMCT）延长、波幅降低,MEP缺失组预后最差,异常组次之,MEP正常组预后最好。结论：MEP可作为评估脑梗死患者预后的一个重要指标。     

Optimising the detection of upper motor neuron function dysfunction in amyotrophic lateral sclerosis—a transcranial magnetic stimulation study

Abstract Evidence of upper motor neuron (UMN) dysfunction is essential in making the diagnosis of amyotrophic lateral sclerosis (ALS). Central motor conduction (CMC) abnormalities detected using transcranial magnetic stimulation (TMS) are presumed to reflect UMN dysfunction. CMC is, however, often normal in patients with classical sporadic ALS. The aim of the study was to determine whether the utility of the CMC measure in ALS could be enhanced. We measured CMC to four pairs of muscles (abductor digiti minimi (ADM), biceps, vastus medialis (VM) and abductor hallucis (AH) in 20 controls and 25 ALS patients. The commonest abnormality detected in the ALS patients was an absent MEP, found in 11 patients (44 %) and in 25 of 200 muscles examined. Studying a minimum of three muscles increased the probability of detecting UMN dysfunction. Weakness in the muscle as well as selecting a distal rather than a proximal muscle was significantly associated with an abnormal CMC. Interside differences in CMC were significantly more pronounced in the patient group. In 30% of patients a significant interside difference in AH CMC time was the sole abnormality, suggesting mild UMN dysfunction on the side with the longer CMC.Financial support from the Wellcome Trust is gratefully acknowledged.     

Motor responses evoked by transcranial magnetic stimulation and peripheral nerve stimulation in the ulnar innervation in amyotrophic lateral sclerosis: the effect of upper and lower motor neuron lesion

We studied the upper (UMN) and lower motor neuron (LMN) innervations of 159 hands from 81 patients with amyotrophic lateral sclerosis (ALS). Eleven patients with various chronic LMN disorders causing weakness in the abductor digiti minimi (ADM) muscle served as LMN controls. Thirty healthy subjects served as normal controls. Cortical motor threshold, central conduction time (CMCT), and motor-evoked response amplitude (MEP) after transcranial magnetic stimulation (TMS) were studied, and the MEP/M wave ratio was calculated. The data was analyzed in the ALS subjects in groups defined by ADM muscle strength and by the presence or absence of clinical signs of UMN involvement.

CMCT was not increased in the ALS or LMN disease groups. The threshold was higher in limbs with both weak ADM muscles and UMN signs. The MEP/M wave amplitude ratio was increased in weak muscles in the ALS patients, notably in limbs with no UMN signs, and also in weak muscles in patients with other chronic LMN disorders. It was frequently decreased in strong muscles. There was no difference between bulbar-onset and limb-onset ALS groups, and there was no correlation between threshold and disease duration. We suggest that expressing the data as an index and utilising the MEP/M wave amplitude ratio as a variable is a sensitive method for detecting UMN abnormality in ALS in particular in early affected muscles.     

Central motor conduction time reveals upper motor neuron involvement masked by lower motor neuron impairment in a significant portion of patients with amyotrophic lateral sclerosis

ObjectiveWe retrospectively investigated the utility of the central motor conduction time (CMCT) in detecting upper motor neuron (UMN) involvements in patients with amyotrophic lateral sclerosis (ALS).MethodsFifty-two ALS patients and 12 disease control patients participated in this study. Surface electromyograms were recorded from the first dorsal interosseous (FDI) and tibialis anterior (TA) muscles. We stimulated the motor cortex, brainstem, and spinal nerve using transcranial magnetic stimulation (TMS) in order to measure the cortical, brainstem, and spinal latencies. We divided the ALS patients into 2 subgroups (with UMN impairment vs. without UMN impairment) and calculated the rates of abnormal CMCT prolongation judged by their comparison with the normal ranges obtained by the measurement in the control patients.ResultsThe CMCTs in the FDI and TA were abnormally prolonged in over 40% of the ALS patients with UMN impairment and in nearly 30% of those without UMN impairment.ConclusionsCMCT shows UMN dysfunction in ALS patients without clinical UMN impairment.SignificanceTMS still has diagnostic utility in a significant portion of ALS patients.     

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.     

Sensitivity of transcranial magnetic stimulation of cortico-bulbar vs. cortico-spinal tract involvement in Amyotrophic Lateral Sclerosis (ALS)

Background An upper motor neuron (UMN) lesion in amyotrophic lateral sclerosis (ALS) is often difficult to identify because clinical signs may be discrete or masked by severe simultaneous LMN lesions. We compared the diagnostic sensitivity of transcranial magnetic stimulation (TMS) to cranial muscles and limb muscles in the detection of UMN lesions. Design We investigated corticobulbar and corticospinal tract function to the tongue/orofacial muscles and abductor digiti minimi/tibial anterior muscles with TMS in 51 patients with ALS to compare the diagnostic yield in the detection of UMN dysfunction. An UMN lesion was assumed when the following were found: the peripheral conduction time and amplitude of the M-wave were within the normal range, the response to cortical stimulation was absent, the TMS evoked/M-wave amplitude ratio was reduced, and the central motor conduction time or the interside difference was delayed (> mean+2.5 SD). Results On the basis of these criteria a UMN lesion to the orofacial muscles was identified in 24 patients (47 %), to the tongue in 27 (53 %), and to the upper and lower limbs in 13 (25 %) and 22 patients (43 %), respectively. Combined abnormalities from all sites increased the diagnostic yield to 39 patients (76 %). TMS of the limb muscles confirmed a UMN lesion in only 15 (54 %) of the 28 patients with clinically confirmed UMN involvement. This number increased to 23 patients (82 %) if tongue and orofacial muscles were taken into acount. Conclusion Our results indicate the early and in most cases subclinical corticobulbar tract involvement of the central motor pathways to the orofacial muscles and tongue in ALS. TMS of the tongue and orofacial muscles had a higher sensitivity in identifying UMN lesions than that of the upper and lower limbs. Received: 13 December 2000, Received in revised form: 15 March 2001, Accepted: 1 April 2001     

The cutaneous silent period as a measure of upper motor neuron dysfunction in amyotrophic lateral sclerosis

ObjectivesWe investigated the cutaneous silent period (CutSP) as a measure of upper motor neuron (UMN) dysfunction in amyotrophic lateral sclerosis.MethodsThe onset latency, duration, and amount of EMG suppression of the CutSP were compared with clinical UMN signs in 24 patients with amyotrophic lateral sclerosis (ALS). UMN signs were quantified using a clinical index and transcranial magnetic stimulation (TMS). Central motor conduction time (CMCT), cortical motor threshold and motor evoked potential amplitudes were assessed as measures of UMN dysfunction. CutSP was studied in abductor digit minimi (ADM) and tibialis anterior (TA) EMG recordings following stimulation of the 5th finger and sural nerves respectively. Non-parametric tests and binomial logistic regression were applied to evaluate the data.ResultsCutSP onset latency was increased in ALS patients, compared to healthy controls, both for ADM and TA muscles. In limbs with clinical UMN signs or abnormal TMS findings, the CutSP onset latency was particularly increased. There was a significant positive correlation between CutSP onset latency and the UMN score in both upper and lower limbs. In TA muscles there was also a negative correlation between CutSP onset latency and EMG suppression. The logistic regression model based on CutSP parameters correctly classified more than 70% of the cases regarding the presence of clinical signs of UMN lesion, in both upper and lower limbs. The results were not significant for TMS.ConclusionWe conclude that upper limb CutSP changes associates with UMN lesion in ALS. This neurophysiological measurement merits further investigation in ALS.     

Quantification of upper motor neuron loss in amyotrophic lateral sclerosis.

OBJECTIVE: To quantitatively estimate upper motor neuron (UMN) loss in ALS. METHODS: We used the recently developed triple stimulation technique (TST) to study corticospinal conduction to 86 abductor digiti minimi muscles of 48 ALS patients. This method employs a collision technique to estimate the proportion of motor units activated by a transcranial magnetic stimulus. At the same time, it yields an estimate of lower motor neuron (LMN) integrity. RESULTS: The TST disclosed and quantified central conduction failures attributable to UMN loss in 38 sides of 24 patients (subclinical in 15 sides), whereas conventional motor evoked potentials detected abnormalities in only 18 sides of 12 patients (subclinical in two sides). The increased sensitivity of the TST to detect UMN dysfunction was particularly observed in early cases. Increased central motor conduction times (CMCT) occurred exclusively in sides with conduction failure. In sides with clinical UMN syndromes, the TST response size (but not the CMCT) correlated with the muscle weakness. In sides with clinical LMN syndromes, the size of the peripherally evoked compound muscle action potentials correlated with the muscle weakness. CONCLUSION: The TST is a sensitive method to detect UMN dysfunction in ALS. It allows a quantitative estimate of the UMN loss, which is related to the functional deficit. Therefore, the TST has a considerable impact on diagnostic certainty in many patients. It will be suited to follow the disease progression and therapeutic trials.     

Sensitivity of electrophysiological tests for upper and lower motor neuron dysfunction in ALS: A six‐month longitudinal study

By following a group of amyotrophic lateral sclerosis (ALS) patients longitudinally using lower motor neuron (LMN) and upper motor neuron (UMN) markers of dysfunction it may be possible to better understand the functional relationships between these motor systems in this disease. We used neurophysiological techniques to follow UMN and LMN dysfunction in a group of 28 patients with ALS, in comparison with the ALS functional rating scale (ALS‐FRS) score and the forced vital capacity (FVC). We used motor unit number estimation (MUNE), compound muscle action potential (CMAP) amplitude, and the Neurophysiological Index (NI) to quantify the LMN disorder, and transcranial motor stimulation to study cortical motor threshold, motor‐evoked response amplitude, central motor conduction time, and cortical silent period (CSP). The patients were studied shortly after diagnosis and then 6 months later, using both abductor digiti minimi muscles (ADM); ADM strength was initially >MRC 3 (Medical Research Council, UK). The NI and MUNE changed more than any other variable. CSP increased by about 30%, a change more marked than the slight increase observed in the cortical motor threshold (9%). The normal increase of CSP after acute muscle fatigue was preserved during disease progression. The CSP increase correlated with the MUNE rate of decay but not to the NI reduction, perhaps because NI includes F‐wave frequency in itscalculation. There was no definite correlation between UMN and LMNdysfunction or progression, but there was a link between CSP and LMN changes in ALS. The CSP may be a useful variable in following UMN dysfunction in clinical practice and in clinical trials. Muscle Nerve, 2010