Single fiber electromyography of extraocular muscles: A sensitive method for the diagnosis of ocular myasthenia gravis

We performed single fiber electromyography (SFEMG) in the superior rectus and levator palpebralis (SR-LP) muscles of 17 patients with pure ocular myasthenia gravis (MG) and 9 controls. Thirteen patients were also assessed with SFEMG in the orbicularis oculi (OO) muscle. All the MG patients but none of the control subjects showed abnormal SFEMG jitter in the SR-LP muscles. On the other hand, only 62% of the MG patients had abnormal SFEMG jitter in the OO muscle. The procedure was well tolerated by the patients, and complications were minor. We conclude that SFEMG of the SR–LP muscles is a safe and highly sensitive technique for the diagnosis of ocular MG. © 1995 John Wiley & Sons, Inc.     

Correlation between a patient-derived functional questionnaire and abnormal neuromuscular transmission in Myasthenia Gravis patients.

OBJECTIVE: To correlate the patient-derived physical function score, as measured by the disease specific Myasthenia Gravis Questionnaire (MGQ), and the score obtained with commonly used generic 36-item questionnaire, the Short-Form health survey (SF-36), with the degree of abnormal neuromuscular transmission measured by Single-Fibre EMG (SFEMG) and repetitive nerve stimulation (RNS) in Myasthenia Gravis (MG) patients. METHODS: SFEMG and RNS were performed in the deltoid muscle and SFEMG was also performed in the orbicularis oculi muscle in 45 MG patients. The patients were asked to fill out the MGQ, which has been translated and validated into Swedish, and the SF-36. The sum of MGQ items generates a global MGQ score. Items are also divided into 3 muscle specific domains: generalized, bulbar and ocular. RESULTS: The global MGQ score and generalized domain score of MGQ were strongly correlated with the degree of abnormal neuromuscular transmission, as measured by SFEMG in the deltoid muscle. Scores from ocular and bulbar domains of MGQ were not related to neurophysiological findings. Physical composite scores of SF-36 correlated significantly with the abnormal SFEMG findings in the deltoid. CONCLUSIONS: The degree of disturbed neuromuscular transmission in a proximal limb muscle correlates with a patient's subjective experience of generalized myasthenic dysfunction. SIGNIFICANCE: The observed correlation adds a new dimension to neurophysiological examinations in patients with MG.     

Repetitive nerve stimulation in myasthenia gravis—relative sensitivity of different muscles

Objective: To correlate repetitive nerve stimulation (RNS) decrement in different muscles with the predominant clinical presentation in myasthenia gravis (MG), and to study single fibre EMG (SFEMG) sensitivity in ocular MG.Methods: Sixty-nine, untreated, consecutive patients suspected for MG were observed prospectively for a minimum of 6 months. Those who improved on medical treatment were diagnosed as MG. The others, in whom the neurophysiological studies were normal and that did not improve on medical treatment served as a control group, from which normative data for RNS and SFEMG was obtained. The MG patients were further classified in 3 subgroups according to the predominant clinical presentation: group I (ocular); group b (bulbar); and group a (axial). We performed RNS in nasalis, trapezius, anconeus, and abductor digiti minimi. All patients with ocular MG underwent jitter determination of the orbicularis oculi muscle.Results: Thirty-seven patients were diagnosed as MG (group I, 15; group b, 13; group a, 9). In group I, RNS was abnormal in 33% of the patients. RNS studies disclosed at least one abnormal muscle response in every patient in groups a and b. Trapezius was significantly more sensitive in group a, and anconeus and nasalis in group b (P<0.01). Jitter was abnormal in all patients in group I, and the most sensitive parameter was an increased number of unstable pairs, 100%.Conclusions: Based on these observations, we recommend that a shoulder muscle, as the trapezius, should be studied first in the limb-axial presentation of MG, and the anconeus–nasalis muscles in predominant bulbar MG. In ocular MG, RNS is not sensitive and jitter should be performed in facial muscles.Significance: This paper shows the unequal sensitivity of several muscles to RNS in different forms of MG.     

Stimulated single-fiber EMG of the frontalis and orbicularis oculi muscles in ocular myasthenia gravis

We performed single-fiber electromyography by axonal stimulation (stimulated SFEMG) of the frontalis and orbicularis oculi muscles of 20 patients with ocular myasthenia gravis (OM) and 46 controls. In controls, mean consecutive differences (MCD) ranged from 5 to 55 micros (average, 14.7 +/- 2.8 micros) in the frontalis and from 4 to 56 micros (average, 12.56 +/- 2.19 micros) in orbicularis oculi. The mean MCD of individual muscle potentials (MPs) was 14.6 +/- 6.8 micros in frontalis and 12.68 +/- 6.10 micros in orbicularis oculi. In the OM patients, the mean MCD was 43.85 +/- 25.18 micros in the frontalis and 69.85 +/- 29.55 micros in orbicularis oculi (P < 0.0001), and the number of MPs with altered MCD was 7.15 +/- 4.66 (range, 1-18) and 12.65 +/- 4.90 (range, 6-21), respectively (P < 0.0001). We conclude that stimulated SFEMG of the orbicularis oculi muscle is more sensitive for the diagnosis of OM than of the frontalis muscle.     

Repetitive nerve stimulation of facial muscles in MuSK antibody-positive myasthenia gravis

To better define electrophysiological abnormalities in myasthenia gravis (MG) patients with muscle-specific tyrosine kinase (MuSK) antibodies (Ab), we compared electrophysiological features of 14 MuSK Ab-positive, 73 acetylcholine receptor antibody (AChR Ab)-positive, and 22 MuSK and AChR Ab-negative (seronegative) patients with generalized disease. Repetitive nerve stimulation (RNS) abnormalities were observed in 86% of MuSK Ab-positive and 82% of AChR Ab-positive patients but in only 55% of seronegative patients. RNS decrements in the orbicularis oculi were more common and severe in the MuSK Ab-positive patients than the other two groups. Single-fiber electromyography (SFEMG) of the extensor digitorum communis was abnormal in 90% of MuSK Ab-positive patients. The high frequency of RNS abnormalities in facial muscles in the MuSK Ab-positive population reflects the propensity for facial muscle involvement in this form of MG and emphasizes the importance of including facial muscles in RNS protocols when evaluating these patients.     

Repetitive hypoglossal nerve stimulation in myasthenia gravis.

OBJECTIVES: To assess the diagnostic efficacy of repetitive nerve stimulation (RNS) of the hypoglossal nerve in patients with myasthenia gravis (MG) and bulbar symptoms (dysphagia, dysarthria). METHODS: Twenty patients with MG and 25 normal controls had RNS of the hypoglossal nerve. All patients also had single fibre electromyography (SFEMG) of the orbicularis oculi and RNS with recordings of the nasalis, trapezius and abductor pollicis brevis muscles. RESULTS: All patients had positive SFEMG studies. Nine patients with bulbar symptoms had positive hypoglossal RNS, including 3 with negative RNS recordings in other muscles. Eleven patients with no bulbar symptoms showed negative hypoglossal RNS, including two with positive RNS recordings from other muscles. CONCLUSIONS: Abnormal RNS of the hypoglossal nerve correlates well with bulbar dysfunction and further characterises the extent of neuromuscular transmission defect in MG patients.     

神经电生理检查判断眼肌型重症肌无力的敏感度分析

目的：分析眼肌型重症肌无力（OMG）患者的神经电生理特点,为临床诊断提供有价值的依据.方法：对42例临床诊断为OMG患者进行单纤维肌电图、重复神经电刺激和肌电图检测.结果：伸指总肌的单纤维肌电图34例异常,重复电刺激异常23例;肌电图示14例肌源性损害.结论：OMG患者单纤维肌电图是一种敏感度较高的检测方法,其次为重复神经电刺激,其肌肉检测阳性率高低依次为眼轮匝肌、肱二头肌及小指展肌.     

Reliability of SFEMG in diagnosing myasthenia gravis: Sensitivity and specificity calculated on 100 prospective cases

ObjectiveThe study aimed to determine the utility of single-fibre electromyography (SFEMG) in the diagnosis of myasthenia gravis (MG) in subjects with a clinical suspicion of the disease.MethodsWe performed a prospective, single-blinded study on 100 consecutive patients. SFEMG was not considered a criterion in making the MG diagnosis. For all cases, a different physician than the one performing SFEMG made the diagnosis of MG. All subjects underwent standard SFEMG of a single muscle, the orbicularis oculi.ResultsSFEMG was abnormal in 67 of 100 patients. A final diagnosis of definite MG was made in 54 patients (30 men/24 women). SFEMG was positive in 53 of 54 patients diagnosed with MG. The sensitivity of SFEMG in diagnosing MG was 98% (95% CI: 0.94–1.02), while the specificity was 70% (95% CI: 0.54–0.86), with a positive predictive value of 79% (95% CI: 0.74–0.79) and a negative predictive value of 97% (95% CI: 0.94–0.99).ConclusionsIn this cohort of patients, normal SFEMG findings were unlikely to occur in patients with MG.SignificanceSFEMG is not a confirmatory test for the diagnosis of MG, but it has a high negative predictive value in identifying patients without MG.     

Diagnostic sensitivity of the laboratory tests in myasthenia gravis.

The diagnostic sensitivity of three laboratory tests [serum antiacetylcholine receptor antibody (AChR-ab) assay, the repetitive nerve stimulation (RNS) test, and, the single fiber EMG (SFEMG)] for myasthenia gravis (MG) was compared in 120 patients. In all cases, at least one of the tests was abnormal. SFEMG was the most sensitive test, being abnormal in 92% of cases, followed by the RNS test (77%) and the AChR-ab assay (73%). SFEMG was abnormal in all cases with negative AChR-ab and RNS tests, in 97% of cases with negative AChR-ab assay, in 89% of cases with negative RNS test, and in 89% of cases with mild MG. We conclude that one of these three tests is abnormal in all cases of MG, and that the SFEMG is most sensitive in the diagnosis of MG.     

New strategy for improving the diagnostic sensitivity of repetitive nerve stimulation in myasthenia gravis

Introduction: The diagnostic sensitivity of repetitive nerve stimulation (RNS) in patients with myasthenia gravis (MG) varies as a function of the number of muscles or the choice of muscles studied. Methods: By exploring 12 muscles bilaterally, we evaluated the global sensitivity of RNS at rest, the sensitivity in different clinical forms, and the sensitivity of different combinations of muscles studied. Results: The global sensitivity of RNS was 82%, and specificity was 100%. The sensitivity in the MG subgroups was as follows: ocular (O) = 67%; oculobulbar (OB) = 86%; and generalized (G) = 89%. The most sensitive muscles were the anconeus in group O, orbicularis oculi (OO) or nasalis in group OB, and the trapezius in group G. Maximum sensitivity was obtained by exploring OO, trapezius, and anconeus bilaterally. Conclusions: We recommend bilateral exploration of at least 3 muscles, a facial muscle, trapezius, and anconeus. Muscle Nerve 55 : 532–538, 2017     

Single fiber EMG as a prognostic tool in myasthenia gravis

Introduction: Single fiber electromyography (SFEMG) is the most sensitive diagnostic tool for diagnosis of myasthenia gravis (MG). Its prognostic value is not known. Methods: We retrospectively analyzed the clinical course of 232 MG patients who presented with only mild symptoms and had SFEMG of the orbicularis oculi muscle. We correlated their SFEMG results with the severity of their later clinical course. Results: During the observation period 39 patients (17%) developed severe disease exacerbations, and 193 (83%) remained stable. Patients with severe disease exacerbation had a significantly higher mean jitter value (P < 0.0001), a greater percentage of fibers with increased jitter (P < 0.0001), and/or impulse blocking (P < 0.0001) on SFEMG. Conclusions: The extent of the SFEMG abnormalities in this study correlated with the later clinical course of MG. Muscle Nerve 54 : 1034–1040, 2016     

Acute ophthalmoparesis in the anti-GQ1b antibody syndrome: electrophysiological evidence of neuromuscular transmission defect in the orbicularis oculi

OBJECTIVE: To prospectively study anti-GQ1b antibody positive cases of acute ophthalmoparesis (AO) clinically and electrophysiologically. METHODS: Nine consecutive cases presenting with predominantly acute ophthalmoplegia were assessed clinically and had stimulated single fibre electromyography (SFEMG) of the orbicularis oculi at presentation. All had magnetic resonance imaging brain scans and anti-GQ1b antibody titres determined. RESULTS: Four cases had elevated anti-GQ1b antibody titres and abnormal SFEMG studies, which improved in tandem with clinical recovery over three months. Five other anti-GQ1b antibody negative cases were diagnosed as diabetic related cranial neuropathy, idiopathic cranial neuropathy, ocular myasthenia gravis, and Tolosa-Hunt syndrome. All five cases showed complete recovery over a three month period. CONCLUSIONS: This study demonstrated electrophysiologically the dynamic improvement of neuromuscular transmission of anti-GQ1b antibody positive cases of AO, in tandem with clinical recovery. SFEMG is of value in differentiating weakness due to neuromuscular transmission defect from neuropathy in these clinical situations.     

胸腺切除术对重症肌无力患者电生理学指标和临床评分的影响

目的评价胸腺切除术对重症肌无力(MG)的近期疗效。方法对15例MG在胸腺切除术前(平均9天)及术后(平均11天)依次进行了临床绝对评分、右侧腋神经低频重复电刺激(RNS)和右侧伸指总肌单纤维肌电图(SFEMG)检查。结果手术后SFEMG和RNS异常率分别为100%和73%,手术后平均颤抖(jitter)值、异常电位对比例、阻滞电位对比例和临床绝对评分都较手术前有显著的降低;病理为胸腺瘤5例,手术前后平均jitter值、阻滞电位对比例和临床绝对评分均无显著差异,胸腺增生9例,手术后阻滞电位对比例和临床绝对评分较术前显著减低。结论MG患者胸腺切除术后在临床表现和电生理学指标上都有一定程度的改善,但此时大多数患者仍有临床症状和电生理学指标的异常,需要进一步的免疫调节治疗;MG并发胸腺瘤者可能手术后近期疗效差,并发胸腺增生者可能疗效相对较好。     

Diagnostic utility of stimulated single-fiber electromyography of the orbicularis oculi muscle in patients with suspected ocular myasthenia

Stimulated single-fiber electromyography (SSFEMG) is a valuable diagnostic tool in cases of myasthenia gravis with limited disease. From 1990 to 2008 SSFEMG in the orbicularis oculi muscle (OO) was performed in a cohort of 456 patients referred with clinical suspicion of myasthenia gravis (MG) and exclusively ocular symptoms. A diagnosis of MG was made on clinical grounds in 103 patients. In this patient cohort, the specificity of SSFEMG for myasthenia was 97%, and the sensitivity was 80%. MG patients with a normal SSFEMG had a benign clinical course. This study confirms a high specificity and sensitivity of SSFEMG when it is performed on patients suspected to have ocular MG. In such patients, a normal SSFEMG of the OO predicts a benign clinical course.     

Single fiber EMG in a congenital myasthenic syndrome associated with facial malformations

Six patients with a newly described genetic syndrome in Iraqi and Iranian Jews of congenital myasthenia associated with facial malformations were studied with voluntary and stimulation single fiber EMG (SFEMG). Voluntary SFEMG revealed abnormal jitter in all patients in both extensor digitorum communis (EDC) and orbicularis oculi (OOC) muscles, though much smaller in the clinically unaffected EDC. SFEMG study of OOC muscle by axonal stimulation at rates from 1 to 48 Hz showed the most increased jitter at the highest stimulation frequencies in the majority of end-plates, one-third of which showed maximal jitter at intermediate rates. These results may suggest a postsynaptic abnormality as the underlying cause for the neuromuscular transmission defect, and demonstrate the usefulness of SFEMG in the diagnosis of congenital myasthenia. © 1993 John Wiley & Sons, Inc.     

Single-fiber electromyography of masseter muscle in myasthenia gravis

Jitter after axonal microstimulation in the masseter muscle was studied in 30 consecutive patients (12 women) with myasthenia gravis (MG). Patients' mean age was 42.3 (12-75), median disease duration was 3 months (1-72), and onset was ocular (15 cases), oculobulbar (7), bulbar (6), or generalized (2). There were 23 newly-diagnosed patients. Nine cases developed purely ocular MG and 21 cases developed generalized MG. In the latter group, five subjects had a rapidly progressive course and 16 subjects had stable or well-controlled disease (MGFA grade 2-3). Six patients did not have circulating anti-acetylcholine receptor antibodies. Masseter single-fiber electromyography (SFEMG) was abnormal in 6 of 9 ocular MG patients and in all generalized cases (overall sensitivity 27 of 30 cases or 90%; confidence interval 79.3%-100.0% at P = 0.95). Masseter should be considered for SFEMG in diagnosis of MG, especially in cases with bulbar onset.     

Quantitative EMG of facial muscles in myasthenia patients with MuSK antibodies.

OBJECTIVE: Our aim was to study the pathophysiological process leading to facial muscle atrophy in 13 patients with MuSK antibody positive myasthenia gravis (MuSK-MG), and to compare with findings from 12 acetylcholine receptor antibody positive myasthenia patients (AChR-MG), selected because they suffered from the same degree of disease severity and required similar treatment. METHODS: Motor unit action potential (MUAP) and interference pattern analysis from orbicularis oculi (O oculi) and orbicularis oris (O oris) muscles were studied using a concentric needle electrode, and compared with findings in 20 normal subjects, 6 patients receiving botulinum toxin injections (representing a neurogenic model) and 6 patients with a muscle dystrophy (representing a myopathic model). The techniques and control data have been reported previously. RESULTS: The mean MUAP durations for O oculi and O oris were significantly reduced (p<0.001) in both MG cohorts when compared with healthy subjects, and were similar to those in the myopathic control group. They were significantly different from those obtained from the neurogenic control group (p<0.001 for both O oculi and O oris). The MUAP findings in O oculi occurred independently from neuromuscular blocking on single fibre EMG (SFEMG) in the same muscle. On turns amplitude analysis (TAA), 50% of MuSK-MG patients and 42% of AChR-MG patients had a pattern in O oculi which was similar to that in the myopathic control group, and 62% of MuSK-MG patients and 50% of AChR-MG patients had a pattern in O oris that was also similar to that in the myopathic control group. The TAA findings for O oculi and O oris in both MG cohorts were different from those obtained from the neurogenic control group. CONCLUSIONS: Facial muscle atrophy in MuSK-MG patients is not neurogenic and the pathophysiological changes are akin to a myopathic process. The selected AChR-MG patients also show evidence of a similar pathophysiological process in the facial muscles albeit to a lesser degree. SIGNIFICANCE: We propose that muscle atrophy in MuSK-MG is a myopathic process consisting of either muscle fibre shrinkage or loss of muscle fibres from motor units. The duration of disease and long-term steroid treatment may be further contributory factors.     

中国南方地区20例肌肉特异性酪氨酸激酶抗体阳性重症肌无力患者临床特点分析

目的总结中国南方地区20例肌肉特异性酪氨酸激酶抗体阳性(MuSK+)的重症肌无力(MG)患者的临床特点。方法纳入2016年10月至2019年6月就诊于复旦大学附属华山医院的MuSK+-MG患者20例,回顾性分析患者的人口学资料(性别、年龄,等)、临床特点(起病年龄、受累肌群、临床分型和病程,等)及电生理特点和治疗药物。结果 20例MuSK+-MG患者中女性多见(75%),20~29岁为起病高峰(45%)。首发临床分型以OssermanⅡb型(55%)及MGFAⅡb型(45%)为主,以延髓肌(45%)及眼外肌(35%)受累起病常见,65%患者肌群受累进展在2个月以内。70%患者存在症状每日波动及新斯的明试验阳性。低频重复电刺激阳性率为75%,其中眼轮匝肌睑部阳性率最高(75%),斜方肌重复电刺激阳性率最低(10%),25%患者可见重复复合肌肉动作电位,22.2%患者可见二联束颤。80%患者使用糖皮质激素治疗。结论中国南方地区MuSK+-MG以女性多见、起病年龄多在20~29岁、波动性明显、延髓肌易受累、进展迅速和眼轮匝肌睑部重复性神经电刺激阳性率高的队列特征。     

Single fiber EMG in the frontalis muscle in ocular myasthenia: specificity and sensitivity.

Patients (n = 41) with isolated weakness of the eyelids or extraocular muscles, who had been referred for single fiber electromyography (SFEMG), were followed up after 4 to 24 months, At follow-up the patients were classified as "definite ocular myasthenia gravis" (MG), "definite other diagnosis," or "no definite diagnosis" on the basis of the completed investigations and subsequent course. The original SFEMG findings in the frontalis muscle were then reviewed. The specificity and sensitivity of SFEMG for "definite ocular MG" could be maximized by using as criteria for abnormality greater than 8/20 pairs with jitter greater than 45 microseconds, or a mean jitter of 20 pairs of greater than 50 microseconds. Patients with abnormal SFEMG according to these criteria have MG, and are likely to require treatment in the immediate future. Patients who have normal SFEMG according to these criteria (and no other demonstrated disorder) may have MG, but it is so mild that they are unlikely to require treatment. Two patients whose final diagnosis was progressive external ophthalmoplegia had normal SFEMG according to these criteria.     

Stimulated single-fiber electromyography in Lambert-Eaton myasthenic syndrome.

The Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder of neuromuscular transmission. Electrodiagnosis is confirmed by an increase in compound muscle action potential amplitude during high-frequency repetitive nerve stimulation or following brief exercise. We describe the results of stimulated single-fiber electromyography in 4 patients with disorders of neuromuscular transmission: LEMS (2), LEMS/myasthenia gravis (MG) overlap (1), and MG (1). Stimulated SFEMG was performed in the extensor digitorum communis muscle with axonal intramuscular suprathreshold stimulation at low and high rates. In all 4 patients, a rate dependence of jitter was found. In LEMS and LEMS/MG, jitter and blocking improved with high stimulation rates, as compared with the opposite effect in MG. We conclude that stimulated SFEMG is a valuable technique in the diagnosis of LEMS.