Electromyography (EMG) accuracy compared to muscle biopsy in childhood

Reports show wide variability of electromyography (EMG) in detecting pediatric neuromuscular disorders. The study's aim was to determine EMG/nerve conduction study accuracy compared to muscle biopsy and final clinical diagnosis, and sensitivity for myopathic motor unit potential detection in childhood. Of 550 EMG/nerve conduction studies performed by the same examiner from a pediatric neuromuscular service, 27 children (ages 6 days to 16 years [10 boys; M:F, 1:1.7]) with muscle biopsies and final clinical diagnoses were compared retrospectively. Final clinical diagnoses were congenital myopathies (5 of 27,18%), nonspecific myopathies (biopsy myopathic, final diagnosis uncertain; 6 of 27, 22%), congenital myasthenic syndrome (3 of 27, 11%), juvenile myasthenia gravis (1 of 27, 4%), arthrogryposis multiplex congenita (2 of 27, 7%), hereditary motor and sensory neuropathy (1 of 27, 4%), bilateral peroneal neuropathies (1 of 27, 4%), and normal (8 of 27, 30%). There were no muscular dystrophy or spinal muscular atrophy patients. EMG/nerve conduction studies had a 74% agreement with final clinical diagnoses and 100% agreement in neurogenic, neuromuscular junction, and normal categories. Muscle biopsies concurred with final diagnoses in 87%, and 100% in myopathic and normal categories. In congenital myasthenic syndrome, muscle biopsies showed mild variation in fiber size in 2 of 3 children and were normal in 1 of 3. EMG sensitivity for detecting myopathic motor unit potentials in myopathies was 4 of 11 (36%), greater over 2 years of age (3 of 4, 75%), compared to infants less than 2 years (1 of 7, 14%), not statistically significant (P = .0879). EMGs false-negative for myopathy in infants < 2 years of age were frequently neurogenic (3 of 6, 50%). In congenital myopathies EMG detected myopathic motor unit potentials in 40%, with false-negative results neurogenic (20%) or normal (40%). Because our study has no additional tests for active myopathies, for example Duchenne muscular dystrophy genetic testing, our sensitivity for myopathies is lower than if we used a more global view. In conclusion, EMG detection rate of myopathic motor unit potentials at a young age was low, improving in children over 2 years of age. In neurogenic and neuromuscular junction disorders, the EMG has a very high detection rate. In children with mild to moderate neurogenic EMG findings and normal nerve conduction, a myopathy should always be considered.     

Muscle ultrasonography and electromyography correlation for evaluation of floppy infants

Floppiness in an infant may have a number of different etiologies from disorders of the brain to spinal cord lesions, neuropathies, neuromuscular junction disorders and myopathies.In this study we aimed to investigate the correlation of muscle ultrasonography (US) and electromyography (EMG) in the diagnosis of floppy infants.The study encompassed 41 floppy infants aged 2-24 months. The muscle US and EMG examinations were performed without awareness of the clinical diagnosis. The final diagnosis was established by molecular genetic tests or muscle/nerve biopsy. The neurogenic group consisted of 16 infants according to their US and EMG findings. Fifteen of them had spinal muscular atrophy proven by genetic analysis and one had polyneuropathy diagnosed by nerve biopsy. Six infants were in the myopathic group according to their muscle US and EMG results. All of them underwent muscle biopsy and microscopic examination revealed five congenital muscular dystrophy and one glycogen storage disease. In two infants the US and EMG data conflicted. Their biopsies were also insufficient for the diagnosis. Seventeen infants had normal US and EMG findings but pathologic cranial magnetic resonance imaging or metabolic/genetic tests. They were considered in the group of central hypotonia.Our results suggest a high concordance of US and EMG findings in the diagnostic work-up of neurogenic and myopathic disorders.     

Electrodiagnostic approach to the patient with suspected neuromuscular junction disorder

The electrodiagnostic (EDX) examination in patients with suspected Neuromuscular Junction (NMJ) disorder constitutes the most advanced and complex type of EDX studies. Understanding the anatomy and physiology of neuromuscular transmission is prerequisite for the comprehension and planning of EDX studies in patients with suspected NMJ disorders. In addition to routine nerve conduction studies and conventional needle electromyography (EMG), the EDX studies, which are most useful in the diagnosis of NMJ disorders, include repetitive nerve stimulation and single fiber EMG.     

Neuromuscular involvement in vegetative and minimally conscious states following acute brain injury

The aim of this study was to evaluate the presence of neuromuscular involvement in patients in vegetative and minimally conscious states (VS, MCS) following acute brain injury. Twenty-two patients (11 in VS, 11 in MCS) admitted to a rehabilitation department underwent nerve conduction, electromyography (EMG) for spontaneous activity and direct muscle stimulation (DMS). Twenty (90.9%) showed abnormal nerve conduction studies, 19 (86.4%) had spontaneous EMG activity, and 7 (31.8%) had abnormal DMS. The time spent in the intensive care unit correlated significantly with the amplitude of the compound muscle action potential of the ulnar nerve and with the amplitude of the sensory nerve action potential of the sural nerve (p < 0.05). No significant differences were found between patients in VS and MCS. Neurophysiological data indicative of neuromuscular involvement are common in patients in VS and MCS. This study underlines the need to implement strategies to prevent and to treat neuromuscular complications in patients in VS and MCS.     

Neuromuscular ultrasound for evaluation of the diaphragm

Neuromuscular clinicians are often asked to evaluate the diaphragm for diagnostic and prognostic purposes. Traditionally, this evaluation is accomplished through history, physical exam, fluoroscopic sniff test, nerve conduction studies, and electromyography (EMG). Nerve conduction studies and EMG in this setting are challenging, uncomfortable, and can cause serious complications, such as pneumothorax. Neuromuscular ultrasound has emerged as a non‐invasive technique that can be used in the structural and functional assessment of the diaphragm. In this study we review different techniques for assessing the diaphragm using neuromuscular ultrasound and the application of these techniques to enhance diagnosis and prognosis by neuromuscular clinicians. Muscle Nerve 47:319‐329, 2013     

Neurophysiologic analysis of neuromuscular symptoms in UK Gulf War veterans: a controlled study

BACKGROUND: UK veterans who were deployed to the Gulf in 1990 to 1991 reported higher prevalence of neuromuscular symptoms. OBJECTIVE: To investigate whether these Gulf War-related symptoms were associated with objective evidence of neuromuscular dysfunction. METHODS: Forty-nine Gulf War veterans with more than four neuromuscular symptoms (Gulf-ill), 26 Gulf-well veterans, 13 symptomatic Bosnian veterans (Bosnia-ill), and 22 symptomatic veterans who were not deployed to the Gulf (Era-ill) underwent detailed neurophysiologic assessment: nerve conduction studies, quantitative sensory and autonomic testing, and concentric needle and single-fiber electromyography (EMG). RESULTS: Nerve conduction studies detected carpal tunnel syndrome in two Gulf-ill, two Gulf-well, one Bosnia-ill, and three Era-ill veterans. Ulnar neuropathy was detected in one Gulf-ill and two Era-ill veterans. However, results of detailed nerve conduction studies of the Gulf-ill veterans were comparable with results observed in the other three groups. Quantitative sensory and autonomic assessments also failed to show any specific abnormalities in the Gulf-ill group. Similarly, quantitative assessment of concentric needle and single-fiber EMG detected no chronic denervation or myopathic changes or any abnormalities of neuromuscular transmission in the Gulf-ill veterans. CONCLUSION: Gulf War-related neuromuscular symptoms are not associated with specific impairments of peripheral nerves, neuromuscular junctions, or skeletal muscles.     

Electromyography and biopsy correlation with suggested protocol for evaluation of the floppy infant

Eighty infants with nonarthrogrypotic floppy infant syndrome (FIS) were evaluated between 1979 and 1990. Electromyographic data were correlated with results of muscle and nerve biopsies in 41 of 80 who had concomitant biopsies (38) or other diagnostic analyses (3). A diagnosis was made of Werdnig–Hoffmann disease (WHD) in 15, a congenital infantile polyneuropathy (IPN) in 3, neuromuscular transmission defect (NMTD) in 2, myopathy in 12, and presumed “central” hypotonia in 9. A very positive correlation rate between nerve conduction studies with electromyography and biopsy results was found in 93% (14 of 15) with WHD and 100% in IPN (3 of 3). However, only 4 of 10 infants (40%) with biopsy-proven myopathy had an abnormal EMG. Only once did the results of electromyography and biopsy conflict. © 1994 John Wiley & Sons, Inc.     

Nerve-muscle involvement in a large family with mitochondrial cytopathy: electrophysiological studies.

Thirteen patients with mitochondrial cytopathy were investigated. They represent different generations, ages, stages, and severities of the disease. All were assumed to have the same metabolic defect. The disease is a multisystem disorder with a metabolic defect located at complex 1 in the respiratory chain. Clinically, the disorder gives symptoms such as hearing loss, retinal pigmental degeneration, ataxia, cardiomyopathy, muscular fatiguability and neuropathy. The patients were investigated with nerve conduction studies, concentric needle EMG, SFEMG, and macro EMG examinations. Neurophysiologic studies revealed signs of myopathy in both the younger members and in those with slight muscular symptoms. In the more advanced stages, neuropathic changes of the axonal type were seen as well. Macro EMG was interpreted as indicating muscle fiber membrane abnormalities in the early stages. Single fiber EMG studies indicate that this metabolic defect does not disturb neuromuscular transmission.     

Referral and diagnostic trends in pediatric electromyography in the molecular era

Introduction: Major advances in genetic analysis and neuroimaging have modified the traditional diagnostic approach for neuromuscular disorders. The purpose of this study was to investigate the role of electromyography (EMG) in the molecular era. Methods: We retrospectively surveyed reports of all EMG studies performed at Boston Children's Hospital from 2001 through 2011. Data were collected on study numbers, patient ages, referring provider subspecialty, study indication, electrophysiological diagnosis, and study utility. Results: A total of 2100 studies were performed. The volume increased from ∽160 to ∽250 studies/year. There was a trend toward studying older children. Neurologists, including neuromuscular specialists, constituted the major referral pool, whereas referrals from orthopedics increased steadily. Polyneuropathies followed by mononeuropathies were the most common indications and diagnoses. Fifty‐seven percent of studies were normal. EMG provided meaningful information in 94% of cases. Conclusion: EMG continues to play a cardinal role in the diagnosis of pediatric neuromuscular disorders, although its practice paradigm is evolving. Muscle Nerve 50:244–249, 2014     

Electrodiagnostic testing in neuromuscular disorders

For the past approximately six decades, electrodiagnostic testing orelectrodiagnosis (EDX) has played an increasingly important role in the clinical evaluation of patients who have neuromuscular disorders. This in part is because of a greater understanding of the pathophysiology of these disorders. Also of importance is the development of the techniques themselves, beginning with basic needle electromyography (EMG) and electroneurography (or nerve conduction studies) (NCS) in the 1940s. Today the clinician has a larger menu of testing options, including somatosensory evoked potentials,quantitative EMG, single fiber EMG, and autonomic testing.The advent of computers has added speed and accuracy to testing     

Neurophysiological impairments in multiple sclerosis—Central and peripheral motor pathways

A systematic review of the literature was conducted comparing neurophysiological outcomes in persons with multiple sclerosis (PwMS) to healthy controls (HC), in studies of the central nervous system (CNS) function comprising motor evoked potentials (MEP) elicited by transcranial magnetic stimulation (TMS) and in studies of the peripheral nervous system (PNS) function comprising electroneuronography (ENG) outcomes elicited by peripheral nerve stimulation. Studies comparing neuromuscular function, assessed during maximal voluntary contraction (MVC) of muscle, were included if they reported muscle strength along with muscle activation by use of electromyography (EMG) and/or interpolated twitch technique (ITT). Studies investigating CNS function showed prolonged central motor conduction times, asymmetry of nerve conduction motor pathways, and prolonged latencies in PwMS when compared to HC. Resting motor threshold, amplitude, and cortical silent periods showed conflicting results. CNS findings generally correlated with disabilities. Studies of PNS function showed near significant prolongation in motor latency of the median nerve, reduced nerve conduction velocities in the tibial and peroneal nerves, and decreased compound muscle action potential amplitudes of the tibial nerve in PwMS. ENG findings did not correlate with clinical severity of disabilities. Studies of neuromuscular function showed lower voluntary muscle activation and increased central fatigue in PwMS, whereas EMG showed divergent muscle activation (ie, EMG amplitude) during MVC. When comparing the existing literature on neurophysiological motor examinations in PwMS and HC, consistent and substantial impairments of CNS function were seen in PwMS, whereas impairments of the PNS were less pronounced and inconsistent. In addition, impairments in muscle activation were observed in PwMS.     

Assessment of muscle function in pathology with surface electrode EMG

Surface electromyography (SEMG) had been and is still widely used in kinesiology and fatigue studies. Its use in routine clinical neurophysiology remains limited. Patients are requiring non-invasive EMG, which could also benefit to physicians and health care system looking for tools to evaluate muscle function in neuromuscular diseases as well as for therapeutic trials. This is the aim of our clinical studies. We apply SEMG envelope signal analysis acquired from maximal voluntary contractions, and the related compound muscle action potential (CMAP). These unspecific parameters cannot be used for neuromuscular diseases diagnosis, objective under study by many research teams. In nerve lesions follow-up studies, our SEMG procedure is related to motor clinical progression. We are currently evaluating it in chronic neuromuscular diseases. The respective contribution in neuromuscular disorders of the different neurophysiology techniques has still to be confirmed, and compared to force measurement by manual or quantitative testing, dynamometry, other suggested techniques (spectroscopy, imaging) as well as functional scales.     

Electrophysiologic studies of critically ill patients

Sepsis and critical illness occur as complications of illness, injury, or surgery in approximately 5% of patients in our critical care unit. Clinical evaluation of the nervous system is difficult in this clinical setting, and electrophysiologic studies are therefore quite valuable. Electroencephalography detects encephalopathy and electromyography (EMG) and nerve conduction studies detect neuromuscular disorders at early stages of their development. Thus, septic encephalopathy occurs in almost all patients and critically ill polyneuropathy in at least 50% of such patients. The polyneuropathy is a predominantly distal axonal degeneration of motor and sensory fibers. A catabolic myopathy is also present, but is difficult to detect electrophysiologically. No defect in neuromuscular transmission has so far been demonstrated. Both the encephalopathy and polyneuropathy may be quite severe, but with vigorous management of the sepsis and critical illness complete recovery may occur in the 40% of patients who survive.     

Deriving pediatric nerve conduction normal values in the very young (<3 years)

ObjectiveThis work describes our efforts to obtain nerve conduction studies normal values in a pediatric cohort between birth and 3 years of age using the extrapolated norms or e-norms method. Interpretation of these studies poses major challenges when no reliable normal values can be found in the literature.MethodsThe e-norms method was used to derive a reference range of upper and lower extremity sensory and motor nerve conductions normal values from a pediatric cohort referred to an EMG Laboratory for nerve conduction studies.ResultsE-norms were calculated for Median, Ulnar, Superficial Peroneal, Sural, and Medial Plantar sensory studies, and for Median, Ulnar, Peroneal, and Tibial motor studies.ConclusionsPediatric electrodiagnostic testing is a very challenging undertaking. The ability to obtain and use normal values from the neurophysiologist’s own referral pool adds great value to their diagnostic work-up.SignificanceEMG and nerve conduction studies can yield invaluable information in the diagnostic work-up of young infants. Using the e-norms method improves on the analysis and interpretation of electrophysiological studies in this age group.     

CORRELATION BETWEEN SINGLE FIBRE EMG JITTER AND ENDPLATE POTENTIALS STUDIED IN MILD EXPERIMENTAL BOTULINUM POISONING

To study the correlation between single fibre EMG jitter and endplate potential amplitude we examined neuromuscular transmission in rats paralysed with botulinum toxin type A with single fibre EMG (SFEMG) in vivo and with intracellular microelectrode techniques in vitro. In muscles that were not completely paralysed SFEMG showed an increased neuromuscular jitter on nerve stimulation and in these muscles endplate potentials were of reduced amplitude. When the nerve stimulation frequency increased the jitter decreased. Intravenous injection of drugs that increase the acetylcholine release and endplate potential amplitude markedly reduced the jitter and the frequency dependence disappeared.     

A comparison of stapedial reflex fatigue with repetitive stimulation and single-fiber EMG in myasthenia gravis

The pattern of stapedial reflex fatigue in response to pulsed acoustic stimulation was measured and compared to results of repetitive nerve stimulation and single-fiber electromyography (EMG) in 89 patients with myasthenia gravis. Studies were also made on 22 patients with other neuromuscular disorders and 40 control subjects with no evidence of neuromuscular impairment. Stapedial reflex fatigue exceeded normal control values in 84% of the patients with myasthenia gravis. Repetitive stimulation and single-fiber EMG measurements were abnormal in 56% and 91% of this same population, respectively. Stapedial reflex abnormalities were most prevalent in patients with mild forms of myasthenia (predominantly ocular or oropharyngeal weakness). Of 22 nonmyasthenic patients with neuromuscular disease tested, 6 had abnormal stapedial reflex fatigue according to our normal values, indicating that this form of testing also detects other diseases of the motor unit. The measurement of stapedial reflex fatigue is painless, is easy to perform, and requires minimal patient cooperation. Due to the relatively high occurrence of abnormal stapedial reflex fatigue in patients with myasthenia gravis, this procedure appears to have considerable potential value in screening and monitoring patients for the presence of defects in neuromuscular transmission.     

AANEM's top five choosing wisely recommendations

Objective: To discuss the American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM)'s Top Five Recommendations in the Choosing Wisely campaign educating physicians and patients on high quality electrodiagnostic and neuromuscular medicine. The AANEM published its Top Five Recommendations in February 2015 in collaboration with the American Board of Internal Medicine Foundation and Consumer Reports. Methods: The Professional Practice Committee (PPC) of the American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) developed this list of recommendations. The PPC members identified areas to be included on this list based on the greatest potential for overuse/misuse, quality improvement, and availability of strong evidence‐based research/support in the literature. Results and Recommendations: Recommendation 1: Don't do a needle electromyography (EMG) test for isolated neck or back pain after a motor vehicle accident, as a needle EMG is unlikely to be helpful. Recommendation 2: Don't perform dermatomal somatosensory evoked potentials (SEPs) for a pinched nerve in the neck or back, as they are an unproven diagnostic procedure. Recommendation 3: Don't do a four limb needle EMG/nerve conduction study (NCS) testing for neck and back pain after trauma. Recommendation 4: Don't do nerve conduction studies without also doing a needle EMG for testing for radiculopathy, a pinched nerve in the neck or back. Recommendation 5: Don't do a magnetic resonance imaging (MRI) scan of the spine or brain for patients with only peripheral neuropathy (without signs or symptoms suggesting a brain or spine disorder).     

Isolated femoral mononeuropathy to the vastus lateralis: EMG and MRI findings

A 16-year-old female track athlete experienced sudden onset of right anterolateral thigh pain, initially thought to be cramping. After 2 months of continued postexercise pain she sought medical evalution. A 3-cm thigh circumference discrepancy was noted on physical exam. She was referred for electromyography (EMG) and magnetic resonance imaging (MRI) studies. Femoral and screening nerve conduction studies were normal. Needle EMG revealed acute neuropathic changes confined to the right vastus lateralis only. These findings were confirmed by MRI, including short T₁ inversion recovery (STIR) sequences. All other medical work-up was normal, including lumber and pelvic MRI, and complete serologic studies. This case represents a unique presentation of an idiopathic femoral mononeuropathy isolated to the vastus lateralis only, which has not been previously reported. The highly unusual anatomical presentation of this case illustrates the emerging complementary usefulness of EMG and MRI in delineating neuromuscular pathology.© 1995 John Wiley &Sons, Inc.     

Single-fiber electromyography in myasthenia gravis.

One-hundred-sixty single-fiber EMG studies of the extensor digitorum communis muscle were performed on 127 patients with myasthenia gravis; 131 demonstrated defective neuromuscular transmission. Jitter determinations in the biceps, deltoid or frontalis muscles increased the diagnostic yield significantly. Evoked-potential EMG studies were abnormal in less than 50 percent of patients in whom they were performed. The most sensitive criterion of abnormality was the percentage of fibers with increased jitter; the sensitivity of the test was enhanced, however, if the mean jitter of the tested muscle was also used as a criterion of abnormality. Since increased jitter may also be seen in primary muscle and nerve disease, these disorders must be excluded by other means before diagnosing myasthenia gravis on the basis of the single-fiber studies.     

Nerve conduction and electromyography studies

Nerve conduction studies (NCS) and electromyography (EMG), often shortened to 'EMGs', are a useful adjunct to clinical examination of the peripheral nervous system and striated skeletal muscle. NCS provide an efficient and rapid method of quantifying nerve conduction velocity (CV) and the amplitude of both sensory nerve action potentials (SNAPs) and compound motor action potentials (cMAPs). The CV reflects speed of propagation of action potentials, by saltatory conduction, along large myelinated axons in a peripheral nerve. The amplitude of SNAPs is in part determined by the number of axons in a sensory nerve, whilst amplitude of cMAPs reflects integrated function of the motor axons, neuromuscular junction and striated muscle. Repetitive nerve stimulation (RNS) can identify defects of neuromuscular junction (NMJ) transmission, pre- or post-synaptic. Needle EMG examination can detect myopathic changes in muscle and signs of denervation. Combinations of these procedures can establish if motor and/or sensory nerve cell bodies or peripheral nerves are damaged (e.g. motor neuronopathy, sensory ganglionopathy or neuropathy), and also indicate if the primary target is the axon or the myelin sheath (i.e. axonal or demyelinating neuropathies). The distribution of nerve damage can be determined as either generalised, multifocal (mononeuropathy multiplex) or focal. The latter often due to compression at the common entrapment sites (such as the carpal tunnel, Guyon's canal, cubital tunnel, radial groove, fibular head and tarsal tunnel, to name but a few of the reported hundred or so 'entrapment neuropathies').