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.     

Stimulated jitter in the masseter muscle: normative values

Nineteen healthy volunteers (median age, 25; range, 18-51 years) were enrolled in a study to obtain normative values for stimulated jitter in the masseter muscle. Axonal microstimulation was performed via a monopolar needle electrode introduced in the masseter 2-2.5 cm above the mandibular angle on the line connecting it with the lateral canthus. The recording single-fiber electromyography (SFEMG) electrode was inserted anteriorly in the twitching area of the muscle. The mean consecutive difference (MCD) values for the 426 endplates studied followed a distribution skewed to the left, with a minimum value of 4.3 micros, maximal 44.7 micros, and a maximum of distribution at 11 micros. Mean pooled MCD measured 16.0 micros, and the mean of mean MCD per study was 13.6 micros. The value of the 95th upper percentile for an individual fiber was 29.3 micros. We suggest an upper normal limit for mean MCD per study of 21 micros and upper normal limit of MCD for individual fibers of 30 micros. The stimulated jitter study of masseter muscle is easy and reliable.     

Depression of neuromuscular transmission in methylmercury-poisoned rats: a glass microelectrode and single fiber electromyography study

Changes in neuromuscular transmission were examined in methylmercury (MeHg)-poisoned rats, given a total oral dose of 60 mg CH₃HgCl at 5 mg/kg/day. A microelectrode study was done on the 21st day. The mean quantal content and mean values of the immediately available pool of ACh in the MeHg-poisoned rats were reduced as compared to those in the control rats, but the mean values for the release probability of ACh did not differ significantly. Stimulation single fiber electromyography (SFEMG) was done on the biceps femoris muscle at 1,5, 10 and 20 Hz on the 28th day. Both a significant and consistent increase in jitter were found at increasing stimulation rates in the MeHg-poisoned rats. The SFEMG findings suggest presynaptic involvement due to accelerated depletion of ACh. We confirmed that neuromuscular transmission is depressed in MeHg-poisoned rats in vivo and in vitro.     

Computer simulation of jitter phenomenon in neuromuscular transmission disorders.

Neuromuscular jitter is a sensitive measure of the safety factor of neuromuscular transmission. Nevertheless, the actual relationship between jitter and the safety factor is unknown because these parameters have not been simultaneously measured. In order to explore the theoretical relationship between them, a computer model of mammalian neuromuscular transmission has been developed. If the safety factor is expressed as the absolute value of the natural logarithm of the probability of a block, the model predicts a double exponential relationship between the safety factor and jitter, except when the percentage of blocks is greater than 90%. In that case, the jitter value decreases. Simulation of acetylcholinesterase inhibition shows that this treatment decreases the neuromuscular transmission blocks more effectively in postsynaptic than in presynaptic disorders. In contrast, when the percentage of blocks is greater than 60%, the jitter value increases in both conditions.     

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.     

Surface stimulation single-fiber electromyography

Stimulation single-fiber electromyography (S-SFEMG) is an alternative method to SFEMG during voluntary contraction (VC-SFEMG). S-SFEMG is preferred to VC-SFEMG in uncooperating patients, children, and patients having severe weakness. Surface stimulation is a viable alternative to needle stimulation in S-SFEMG. In the present study, surface versus needle stimulation of facial nerve has been tested by recording from the orbicularis oculi and frontalis muscles. Jitter values obtained by both methods have been found to be nearly equal. © 1998 John Wiley & Sons, Inc. Muscle Nerve, 21: 118–120, 1998.     

Evaluation of fatigue in Parkinson's disease patients with stimulated single fiber electromyography

OBJECTIVES: Fatigue is a disabling and dopa-resistant symptom in Parkinson's disease (PD). Pathological studies have shown that both peripheral and central cholinergic systems are involved in PD. Electrophysiological investigation showed decremental muscle responses following repetitive nerve stimulation (RNS). We hypothesized that fatigue in PD patients may be secondary to cholinergic defect at the neuromuscular level. MATERIAL AND METHODS: Twenty PD patients with disabling fatigue symptoms were enrolled. We used stimulated single fiber electromyography (s-SFEMG) to evaluate the neuromuscular junction of these patients. For every patient, at least 15 muscle fibers were tested at the rate of 10 Hz with the method described by Trontelj et al. The individual mean consecutive difference (i-MCD) was obtained from 100 constant consecutive single fiber potentials. The i-MCD values in each patient were averaged to obtain the mean MCD (m-MCD). RESULTS: None of the 20 PD patients with disabling fatigue symptoms had an m-MCD over 29 micros or more than 10% of the i-MCD values over 41 micros. The neuromuscular junction was intact in terms of normal jitter (expressed as MCD) and an absence of blocking. CONCLUSION: Our findings indicate that the cholinergic system at the neuromuscular level is not affected in PD patients with fatigue symptoms.     

Stimulated single-fiber electromyography in the rat

We constructed an animal model of stimulated single-fiber electromyography (SFEMG) by testing Wistar rats under anesthesia. Stimuli of 1 Hz were applied to the sciatic nerve through an insulated monopolar needle electrode. Single-fiber action potentials were acquired from the gastrocnemius muscle. Jitter was assessed by the mean consecutive difference (MCD). Eighty-seven fibers were obtained from 12 rats. Their MCDs ranged from 2 to 72 μs (17.7 ± 13.4). Seven of these values were less than or equal to 5 μs, and three exceeded 50 μs. Neuromuscular blocking agents injected into some of the rats induced considerable increases in jitter and blocking. A rat with one fiber with an MCD less than 5 μs also received an injection of curare. The jitter showed the same pattern of increment, evidence that the small jitter was not attributable to direct muscle stimulation. These results show that SFEMG can be used on rats. In addition, jitter reflects the changes in motor end-plate function. The findings also suggest the presence of an extremely high safety factor in rat neuromuscular junctions. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21:482–489, 1998.     

Single‐fiber electromyography of the laryngeal muscles

Single‐fiber electromyography (SFEMG) is useful in the evaluation of disorders of neuromuscular transmission and the assessment of motor unit morphology. Standard EMG techniques are used routinely in the evaluation of laryngeal dysfunction, but the feasibility of laryngeal SFEMG has not been established. We, therefore, performed laryngeal SFEMG in 10 normal individuals to demonstrate the feasibility of the technique and generate preliminary normative data. We also studied 2 patients with amyotrophic lateral sclerosis and 1 patient previously treated with botulinum toxin for comparative purposes. © 1999 John Wiley & Sons, Inc. Muscle Nerve 22: 111–114, 1999     

Determining neuromuscular jitter using a monopolar electrode.

Neuromuscular jitter was determined in the extensor digitorum communis (EDC) of 41 healthy control subjects and 8 patients with myasthenia gravis (MG). Standard single-fiber electromyographic (SFEMG) techniques were used, except that a monopolar electrode (MPE) was substituted for a single-fiber electrode (SFE). In normals, mean jitter for 20 pairs was 22.4 +/- 2.8 microseconds and the mean jitter for an individual pair was 22 +/- 5.6 microseconds. In the age range tested (18 to 49 years), jitter did not change with increasing age. When MPE jitter studies were repeated in 16 normals, mean jitter usually varied by less than 10% (mean 7.4% +/- 6.2%) and remained normal. Jitter was significantly increased in MG compared to normals 73 +/- 56 microseconds versus 22 +/- 5.6 microseconds (F = 149.4, P less than 0.0001). Using an analog pain scale, the discomfort during electromyography using MPE and SFE was compared between the left and right arms in 35 normals. MPEs caused less discomfort than SFEs (P = 0.0031), and were preferred (71% versus 20%) to SFEs (chi 2 = 9.21, P = 0.01). Based on these results, we conclude that MPE determined jitter studies are reproducible, can distinguish between normals and MG, and are associated with less discomfort than SFE.     

Axonal degeneration in the Trembler-j mouse demonstrated by stimulated single-fiber electromyography

The Trembler-j (Tr-j) mouse is a naturally occurring mutant with a point mutation in the peripheral myelin protein-22 gene causing severe peripheral nerve demyelination. It is a genetically homologous murine model for Charcot-Marie-Tooth disease type 1A (CMT 1A). Our prior pilot studies using stimulated single-fiber needle electromyograpy (SSFEMG) showed increased jitter in 60-day-old Tr-j mice compared to age-matched, wildtype animals. The aim of this study was to better elucidate the etiology of increased jitter in Tr-j mice and test the following hypotheses: (1) the increased jitter in Tr-j mice is due to turnover of endplates secondary to axonal degeneration with reinnervation and not to conduction block secondary to demyelination of motor nerve axons; and (2) aging Tr-j mice demonstrate increased jitter and fiber density compared with younger mutant mice due to progressive motor axon loss. SSFEMG studies performed on 60- and 140-day-old mice indicated that average mean consecutive difference (MCD) and fiber density estimates (FDE) were significantly increased in Tr-j mice at both ages compared to age-matched wildtypes. FDE also increased substantially in older mutant mice. Intraperitoneal neostigmine injections produced significant reductions in average MCD in Tr-j mice, suggesting that impaired neuromuscular transmission is an early pathologic feature in these mice and likely reflects distal axonal degeneration. Our findings corroborate our prior pilot study, although in a much larger number of animals across a wider age span. Our study also indicates that SSFEMG, performed in a serial fashion, is a useful, noninvasive method of detecting progressive axon loss in this murine model of CMT 1A. This technique may be a valuable tool to study the affects of genetic or pharmaceutical interventions in murine models of peripheral neuropathy. Muscle Nerve, 2007.     

Decomposition-based quantitative electromyography: methods and initial normative data in five muscles

Quantitative electromyographic (EMG) techniques provide clinically useful information to aid in the diagnosis and follow the course or response to treatment of diseases affecting the motor system. The purpose of this study was to describe a decomposition-based quantitative electromyography method (DQEMG) designed to obtain clinically applicable information relating to motor unit potential (MUP) size and configuration, and motor unit (MU) firing characteristics. Additionally, preliminary normative data were obtained from the deltoid, biceps brachii, first dorsal interosseous, vastus medialis, and tibialis anterior muscles of 13 control subjects. DQEMG was capable of efficiently and accurately extracting MUP data from complex interference patterns during mild to moderate contractions. MUP amplitude, surface-detected MUP (S-MUP) amplitude, MUP duration, number of phases, and MU firing frequencies varied significantly across muscles. The mean parameter values for the individual muscles studied were similar to previous reports based on other quantitative methods. The main advantages of this method are the speed of data acquisition and processing, the ability to obtain MUPs from MUs with low and higher recruitment thresholds, and the ability to obtain both S-MUP or macro-MUP data as well as MU firing rate information.     

单纤维肌电图对糖尿病周围神经病的诊断价值

目的 探讨单纤维肌电图（SFEMG）在糖尿病周围神经病（DPN）中的应用。方法 采用Viking Ⅳ肌电图仪,测定36例2型糖尿病患者指总伸肌的颤抖和纤维密度（FD）,同时进行常规神经传导检测（NCS）并测量空腹血糖和糖化血红蛋白（HbA1C)。结果 颤抖和FD具有相关性,且均与HbA1C呈正相关。18例NCS异常者,颤抖值均超过正常范围（11例伴阻滞）,14例FD增加;18例NCS正常者,7例颤抖值增大（3例伴阻滞）,5例FD增加。结论 颤抖和FD所反映的失神经－神经再支配与代谢状况相关联;SFEMG是DPN早期诊断的敏感手段,可发现糖尿病亚临床周围神经病变。     

Literature review of the usefulness of repetitive nerve stimulation and single fiber EMG in the electrodiagnostic evaluation of patients with suspected myasthenia gravis or Lambert-Eaton myasthenic syndrome

A retrospective literature review of the electrodiagnosis of myasthenia gravis (MG) and Lambert--Eaton myasthenic syndrome (LEMS) through July 1998 was performed for the purpose of generating evidence-based practice parameters. There were 545 articles identified, of which 13 articles met at least three of the six criteria set previously by the American Association of Electrodiagnostic Medicine (AAEM). An additional 21 articles were identified from review articles or the references of these first 13 articles leading to a total of 34 articles. Results of studies utilizing repetitive nerve stimulation (RNS) showed that a 10% decrement in amplitude from the first to fourth or fifth intravolley waveform while stimulating at 2--5 HZ is valid for the diagnosis of MG. The degree of increment needed for the diagnosis of LEMS is at least 25% but most accurate when greater than 100%. Abnormal jitter or impulse blocking are the appropriate criteria for diagnosis of neuromuscular junction (NMJ) disorders when using single fiber electromyography (SFEMG). SFEMG is more sensitive than RNS for the diagnosis of disorders of neuromuscular transmission, but may be less specific and may not be available. Therefore, RNS remains the preferred initial test for MG and LEMS.     

Single-fiber electromyography in neuromuscular disorders: Correlation of muscle histochemistry,single-fiber electromyography,and clinical findings

We performed single-fiber electromyography (SFEMG) and correlated the results with muscle-biopsy histochemistry in 56 patients with various neuromuscular diseases. Increased muscle-fiber density, delineated by SFEMG, was most prominent in diseases of ordinary denervation, namely motor neuron disorders and peripheral neuropathies, and it correlated with histochemical fiber type grouping. Both phenomena reflect denervation followed by reinnervation. In patients with central core disease and rod disease, fiber density was not increased despite massive type I fiber predominance. The normal distribution of type I fiber subtypes in those patients indicated that their fiber predominance was not due to sprouting and reinnervation, but probably to paucity of the type II fibers. In type I fiber hypotrophy with central nuclei, fiber density was increased, perhaps attributable to the small diameter and consequent denser packing of the type I fibers. Fiber density was slightly increased in the majority of patients with acidmaltase deficiency, limb-girdle dystrophy, and polymyositis, in nearly half with mitochondrial myopathy, and in 1 older Duchenne dystrophy patient. In these myopathic disorders, myogenous deinnervation (followed by reinnervation) is one possible explanation. Normal fiber density was present in all patients with muscle phosphorylase deficiency, myotonia congenita, and in the hypokalemic periodic paralysis patients under age 40. © 1994 John Wiley & Sons, Inc.     

Single fiber EMG reference values: A collaborative effort

Single fiber electromyography (SFEMG) measurements of fiber density and jitter are used in the diagnosis of a variety of peripheral nervous system disorders. However, the normal values of these measurements for most muscles and age groups are not well documented in the literature. We present a retrospective and prospective multicenter collection of SFEMG jitter and fiber density data from control subjects obtained for the purpose of defining reference values for many muscles and different ages. The data and calculated upper limits for fiber density, individual pair jitter, and mean jitter are presented for each muscle in tabular and graphical format, for different age groups.     

Standardization of anal sphincter electromyography: normative data.

OBJECTIVES: Electromyography (EMG) of the external anal sphincter (EAS) is important in the evaluation of conus/cauda lesions, the differential diagnosis of parkinsonism and anal incontinence. The aim of our study was to establish normative data in a sufficiently large group of healthy subjects, using a rigorously standardized examination technique. METHODS: Sixty-four subjects (aged 19-83 years) without pelvic or neurological disorders were included. Motor unit potentials (MUPs)/interference pattern (IP) samples were obtained from the EAS using multi-MUP and turn/amplitude analyses, respectively. The effect of age, gender, parity, and constipation on MUP/IP parameters was studied. For MUP parameters the lower/upper limits for mean values, and 'outlier' limits, and for IP parameters normal 'clouds' were calculated. RESULTS: From 112 muscles 15-30 MUPs were sampled. As no effect of evaluated factors on mean values could be demonstrated, common reference values were calculated. Lower/higher limits for mean values were: amplitude 148/661 microV, duration 3.2/7.8 ms, area 87/625 microVms, and number of phases 2. 3/3.7. 'Outlier' limits for individual MUPs were: amplitude 84/1315 microV, duration 1.6/13.8 ms, area 46/1222 microVms, number of phases 2/6. From 95 muscles 2706 IP samples were obtained. CONCLUSIONS: The presented normative data should allow valid quantitative EMG of the EAS muscle in patients.