AAEM minimonograph #44: Diseases associated with motor unit activity

Stiff-man syndrome is due to hyperexcitability of anterior horn cells, possibly related to interference with the synthesis or action of gammaaminobutyric acid. Unexpected acoustic and exteroceptive stimuli produce exaggerated muscle responses. Needle electrode examination of involved muscles yields nonspecific findings and demonstrates involuntary motor unit activity. The appearance and firing pattern of motor units are normal except that agonist and antagonist muscles may contract concurrently. Continuous muscle fiber activity (Isaacs' syndrome) comprises a heterogeneous group of hereditary and acquired disorders that cause hyperexcitability of peripheral nerves. Some are associated with electrophysiologic evidence of peripheral neuropathy and some are not. Repetitive afterdischarges often follow the M-, H-, and F-waves. Needle electrode examination reveals an abnormal pattern of motor unit firing, consisting of myokymic discharges, doublets and multiplets, neuromyotonic discharges, and fasciculations. These abnormalities may occur alone or in combination.     

Motor unit recruitment pattern during low-level static and dynamic contractions

Motor unit (MU) recruitment patterns were studied during dynamic and static contractions at workloads corresponding to 10% of maximal voluntary contraction force. The dynamic contraction consisted of a 20% flexion and extension of the elbow performed with a velocity of 10μ/s. Motor unit potential trains were recorded from the branchial biceps muscle of 6 healthy females using a quadripolar needle electrode and a computerized decomposition program. Properties of the identified MUs were derived from concentric needle EMG. A total of 119 MUs were identified during dynamic contractions, 107 MUs during static anisotonic contractions, and 96 MUs during static isotonic contractions. The main result was that MUs recruited during different contractions showed similar properties and may belong to the same part of the motoneuron pool. This indicates that MU recruitment patterns during dynamic contractions may be almost as stereotypical as during static contractions and may even activate the same MUs.© 1995 John Wiley &Sons, Inc.     

AAEM minimonograph #25: Single-fiber electromyography

Single-fiber electromyography (SFEMG) is a selective recording technique in which a needle electrode with a small recording surface in the side is used to identify action potentials from individual muscle fibers. The SFEMG parameters of greatest clinical use are fiber density (FD) and neuromuscular jitter. FD reflects the local organization of muscle fibers within the motor unit; jitter reflects the safety factor of neuromuscular transmission at individual neuromuscular junctions. SFEMG can be of great value in demonstrating or excluding abnormalities in mild or questionable disease of nerve, muscle, or the neuromuscular junction. The neuromuscular jitter may be measured during nerve stimulation, which is particularly useful in uncooperative patients or when it is desirable to control the firing rate precisely, or during voluntary muscle activation, which is less subject to technical artifact. The SFEMG findings may not be specific to a particular diseases, but they frequently increase understanding of the disease process by demonstrating abnormal neuromuscular transmission or rearrangement of muscle fibers within the motor unit, which complements information from more conventional EMG examinations. © 1996 American Association of Electrodiagnostic Medicine. Published by John Wiley & Sons, Inc.     

Motor unit recruitment during lengthening contractions of human wrist flexors.

The purpose of this study was to revisit the question of recruitment of motor units during lengthening contractions because of conflicting views in the literature on this subject. Motor unit activity was recorded from the flexor carpi radialis muscle of four human subjects to compare the patterns of recruitment during lengthening and isometric contractions. Lengthening contractions were produced either when the subject voluntarily stopped opposing a background load or when an additional load was imposed on the already contracting muscle. In both cases, lengthening of the active muscle was produced at a variety of speeds, from quite slow to "as fast as possible." No differences in recruitment order were observed between isometric and lengthening contractions at any speed of lengthening contraction. It is concluded that all contractions in normal humans recruit motor units in an orderly fashion from small to large, according to the size principle of motor unit recruitment.     

Antigravity posture for analysis of motor unit recruitment: the "45 degree test"

The maximum number of different motor unit action potentials (MUAPs), their firing rates, and total MUAP spikes/second recorded by monopolar needle electrode were determined for the biceps brachii muscle during 45-degree elbow flexion. There were 4.2 +/- 1.6 different MUAPs exceeding 100 microV. Mean firing rate was 10.0 +/- 1.7 Hz, and total MUAP spikes/second were 40.3 +/- 18. Recordings from 16 patients with neurogenic atrophy (NA) and just detectable weakness revealed corresponding values of 3.1 +/- 1.7 different MUAPs, a mean rate of 10.2 +/- 1.5 Hz and 30.6 +/- 19 total MUAP spikes/second, not different from normal. In these patients, increased force of muscle contraction was required to activate high threshold motor units firing at high rates. In each of 4 patients just able to hold the arm against gravity, 1 or 2 "overdriven" motor units firing at a mean rate greater than 20 Hz were recorded. In 8 patients with myopathy and just detectable weakness, greater than 100 total MUAP spikes/second were recorded. Antigravity posture as a reference level of innervation has the advantage that motor unit firing rate is set about that of physiologic tremor (10-13 Hz). Its application was helpful in quantifying recruitment.     

AAEM minimonograph #37: facial and limb myokymia.

Myokymia is a clinical phenomenon associated with characteristic electromyographic activity referred to as myokymic discharges. These are spontaneously generated bursts of individual motor unit potentials with each burst recurring rhythmically or semirhythmically, usually several times per second. It involves facial muscles more commonly than those of the extremities, and is most often seen in association with Guillain-Barré syndrome, multiple sclerosis, radiation plexopathy, pontine tumors, and timber rattlesnake envenomation. An alteration in the biochemical microenvironment of axon membranes at one of the various sites along the motor axon is the likely basis for the altered membrane excitability that underlies the myokymic discharges in most cases. The similarity of these discharges to those seen with hypocalcemic tetany, and the ability to manipulate myokymic discharges by altering serum-ionized Ca++, suggests that decrease in the ionized Ca++ in the microenvironment of the axon may play an important role.     

AAEM minimonograph #46: Neurogenic muscle hypertrophy

Muscle hypertrophy occurs uncommonly in several neurogenic disorders including neuropathies, radiculopathies, spinal muscular atrophy, and post-polio syndrome. Its pathogenesis varies in different circumstances. In the presence of generalized myokymia and neuromyotonia (Isaacs' syndrome), symmetrical hypertrophy appears to be the result of continuous spontaneous electrical stimulation of myofibers and, in some cases, results in type 1 myofiber preponderance. Focal hypertrophy occurring with radiculopathies and mononeuropathies was associated with complex repetitive discharges (CRDs) in approximately half the cases. CRDs may play a role in the pathogenesis of myofiber hypertrophy by continuous myofiber stimulation, but in some cases, with and without CRDs, myofiber hypertrophy may be related to mechanical events. Muscle enlargement seen in old polio appears to involve a significant degree of pseudohypertrophy, although some myofiber hypertrophy occurs. The symmetrical occurrence of hypertrophy in genetically determined disorders, such as spinal muscular atrophy, and hereditary motor and sensory neuropathy types 1 and 2 may have both a genetic and a mechanical basis in addition to pseudohypertrophy in some cases. © 1996 American Association of Electrodiagnostic Medicine. Published by John Wiley & Sons, Inc.     

AAEM minimonograph #38: neuropathies in connective tissue disease.

Neuropathies are common in patients with known or suspected connective tissue disease. A vasculitic mononeuropathy multiplex is often seen in patients initially presenting with polyarteritis nodosa or developing arteritis as a complication of rheumatoid arthritis. However, vasculitic neuropathy may become confluent and present as as distal symmetrical polyneuropathy or occur without systemic necrotizing vasculitis. Distal symmetrical polyneuropathies without associated vasculitis are also common in many connective tissue diseases. Compression neuropathies, especially carpal tunnel syndrome, occur with increased frequency in rheumatoid arthritis. Finally, certain neuropathies may be the major presenting feature of particular connective tissue diseases. For example, trigeminal neuropathy often heralds the onset of systemic sclerosis or mixed connective tissue disease, and sensory neuronopathy may be the initial presenting feature of Sj?gren's syndrome.     

F waves and motor unit size.

M responses and twitch contractions were evoked in single motor units (MUs) of the first dorsal interosseus muscle by intramuscular microstimulation of motor axons. Two-hundred nine MUs were studied in 21 subjects. Thirty-five MUs (17%) showed F waves in addition to M responses. Twitch force was used to provide an indirect measure of MU size; additionally, twitch contraction time was measured. There was no select group of MUs generating F waves with regard to the above contraction parameters. However, four of five MUs with very high twitch forces, above 70 mN, generated F waves. We conclude that MUs of all sizes produce F waves with similar probability. Only few MUs with very strong twitch forces, i.e., very large MUs, may be more subject to F-wave production and may be involved in the generation of the so-called repeater F waves.     

AAEM minimonograph #11: Needle examination in clinical electromyography

The physiologic and histologic principles underlying clinical electromyographic studies are briefly reviewed as an introduction to the normal and abnormal findings in human subjects. Technical aspects of recordings as well as the specific types of discharges and their significance are discussed.     

AAEM minimonograph #45: The early development of electromyography

The use of electricity for therapeutic purposes began in the first century and became more refined as the properties of electricity became more understood. The works of Franklin, Galvani, Volta, and others contributed to this body of knowledge. Development of the string galvanometer, the advent of the vacuum tube, the introduction of concentric needle electrodes, and the development of the cathode-ray oscilloscope occurred during the first half of the 20th century. The science of electromyography and electrodiagnosis grew in its sophistication, leading to the formation of the American Association of Electromyography and Electrodiagnosis (now the American Association of Electrodiagnostic Medicine) with James Golseth, MD, as its first president in 1953. © 1995 John Wiley & Sons, Inc.     

AAEM minimonograph #47: Normative data in electrodiagnostic medicine

This article reviews, without mathematics, the important principles governing the acquisition and use of normative data in electrodiagnostic medicine. Common flaws in neurophysiological normative data include vague clinical criteria for establishing freedom from disease, samples that are too small and inadequately stratified, and application of Gaussian statistics to non-Gaussian variables. Other problematic issues concern the trade-off between permissible false-positivity and false-negativity in defining the limits of normative from sample data, test-retest variability, and the use of multiple independent test measurements in each electrodiagnostic examination. The following standards for normative data are proposed: (1) standardized objective determination of freedom from disease; (2) appropriately large sample of normal subjects; (3) proportional statification of normal subjects for known relevant variables; (4) test of Gaussian fit for application of Gaussian statistics; and (5) data presentation by percentiles when Gaussian fit is in doubt. Many existing normative studies in clinical neurophysiology do not meet these standards. High-quality normative data, readily accessible, is essential for the accurate electrodiagnosis of neuromuscular diseases. © 1997 American Association of Electrodiagnostic Medicine. Published by John Wiley & Sons, Inc.     

AAEM case report #30: Multifocal motor neuropathy

A 73-year-old man with a 16-year history of fasciculations and 15 years of weakness in his right arm was diagnosed with focal motor neuron disease. After 10 years of purely motor symptoms, he developed mild parasthesias although his sensory examination remained normal. Reflexes were reduced or absent in the weak muscles but were normal elsewhere. Nerve conduction was studied in nerves innervating weak muscles and showed severe motor conduction block. Sensory nerve conduction studies were minimally abnormal, showing reduced amplitudes with normal velocities. Based on the clinical picture and the presence of severe motor conduction block, the patient was diagnosed with multifocal motor neuropathy. Treatment with high-dose intravenous immunoglobulin was given with significant improvement in strength and partial resolution of the conduction block. As this case demonstrates, this treatable disorder may occasionally be mistaken for motor neuron disease although the resemblance is only superficial, and it should never be mistaken for amyotrophic lateral sclerosis. Multifocal motor neuropathy is an inflammatory, demyelinating neuropathy which, like chronic inflammatory demyelinating polyneuropathy (CIDP), is probably immune-mediated. It differs from typical CIDP by virtue of a marked predilection for motor axons, a strikingly restricted distribution, and a protracted course. Treatment with high-dose intravenous immunoglobulin is frequently helpful, but other forms of immune manipulation are less effective. © 1996 Gareth J. Parry, MD, FRACP. Published by John Wiley & Sons, Inc.     

AAEM minimonograph #2: Important anomalous innervations of the extremities

Anomalous innervations of the extremities are common and influence the interpretation of electrophysiologic studies in normal patients and those with peripheral nerve lesions. The following anomalous innervations are reviewed: median to ulnar nerve communication; ulnar to median nerve communication; variations in the innervation of intrinsic muscles of the hand; accessory deep peroneal nerve; and tibial to peroneal nerve communication. The electrophysiologic recognition of these anomalies and the manner in which they affect the interpretation of electrodiagnostic studies in various conditions is emphasized.     

AAEM minimonograph #40: Clinical neurophysiology of the respiratory system

Disorders of nerve and muscle are frequent causes of respiratory insufficiency, but current methods often fail to adequately assess the problem. Phrenic nerve conduction and needle electromyography of the diaphragm are of great assistance in identifying the nature and site of the disorder: the various disturbances of central drive, axonal or demyelinating neuropathies of the phrenic nerves, and certain myopathies. These studies may be performed on adults, children, or infants, and in outpatient, general ward, or critical care settings. It is hoped these techniques will stimulate further developments in the field.     

AAEM minimonograph #14: The influence of temperature in clinical neurophysiology.

AAEM MINIMONOGRAPH # 14 Temperature affects biologic and neurophysiologic processes and is, therefore, always well controlled in in vitro experiments. Its role is equally important in the clinical laboratory but has often been neglected. Lower temperature cause slower nerve conduction velocities (NCVs), and increased amplitudes of muscle and nerve potentials. Fibrillations may disappear, and muscle contraction will be slower and weaker. Neuromuscular transmission improves. Somatosensory evoked potentials (SEPs) are similarly vulnerable in the peripheral segments, or with changes in central temperature. As a result, abnormalities are artificially created or existing defects are not detected, resulting in false or missed diagnoses. Control of temperature, albeit somewhat time consuming, will result in greater diagnostic accuracy.     

Tracking motor unit action potentials in the tibialis anterior during fatigue

New surface electromyogram (SEMG) techniques offer the potential to advance knowledge of healthy and diseased motor units. Conduction velocity (CV) estimates, obtained from indwelling electrodes, may provide diagnostic information, but the standard method of CV estimation from SEMG may be of only limited value. We developed a motor unit (MU) tracking algorithm to extract motor unit conduction velocity (MUCV) and motor unit action potential (MUAP) amplitude estimates from SEMG. The technique is designed to provide a noninvasive means of accessing fatigue and recruitment behavior of individual MUs. We have applied this MU tracking algorithm to SEMG data recorded during isometric fatiguing contractions of the tibialis anterior (TA) muscle in nine healthy subjects, at 30%-40% maximum voluntary contraction (MVC). The results reveal that MUCVs and MUAP amplitudes of individual MUs can be estimated and tracked across time. Time-related changes in the MU population may also be monitored. Thus, the SEMG technique employed provides insight into the behavior of the underlying muscle at the MU level by noninvasive means.     

AAEM minimonograph #43: Neuromuscular problems in the performing arts

Over the past 10–15 years, there has been increasing interest in the health problems of performing artists. In this review, I will discuss the major playing-related disorders seen in instrumental musicians. Among the 672 instrumentalists evaluated, the major diagnoses identified included musculoskeletal disorders in 64%, peripheral nerve problems in 22.5%. and focal dystonia in 7%. Sixty percent of instrumentalists were female although males predominate in the group with focal dystonia. The average age of those evaluated was 32 years. Among musculoskeletal disorders overuse syndrome is the most common. Frequent peripheral nerve disorders include thoracic outlet syndrome, carpal tunnel syndrome, and ulnar neuropathy. A characteristic distribution of symptoms and signs is identified for each instrument group. Electrodiagnostic studies are an important part of the evaluation of these disorders. With carefully designed treatment, the majority of instrumental musicians can be returned to full and pain-free playing activities. The success rate is highest in some entrapment neuropathies but remains low in focal dystonia. © 1994 John Wiley & Sons, Inc.     

AAEM minimonograph #26: The electrodiagnosis of carpal tunnel syndrome

The electrodiagnosis of carpal tunnel syndrome (CTS) is reviewed, including discussions of old and new techniques of motor and sensory nerve conduction, anomalous innervation, and needle electrode examination. A variety of sensitive nerve conduction studies (NCSs) are available for the evaluation of a patient with suspected CTS. For any particular patient, the NCS method chosen by the clinical neurophysiologist may vary for a numer of reasons, including the severity of the deficit and the presence of superimposed conditions. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20: 1477–1486, 1997     

AAEM minimonograph #35: Clinical experience with transcranial magnetic stimulation

We elicited motor evoked potentials (MEPs) using transcortical magnetic stimulation in 150 control subjects aged 14 to 85 years and 275 patients with a variety of diseases. There were no significant side effects. Cortex-to-target muscle latencies measured 20.2 +/- 1.6 ms (thenar), 14.2 +/- 1.7 ms (extensor digitorum communis), 9.4 +/- 1.7 ms (biceps), and 27.2 +/- 2.9 ms (tibialis anterior). Central motor delay between the cortex and the C-7 and L-5 measured 6.7 +/- 1.2 ms and 13.1 +/- 3.8 ms, respectively. Mean spinal cord motor conduction velocity measured 65.4 m/s. MEP amplitude expressed as a percentage of the maximum M wave was never less than 20% of the M wave. A value of less than 10% is considered abnormal. MEP latency increases linearly with age and central motor delay is longer in older subjects. Compound muscle action potentials and absolute MEP amplitudes decreased linearly with age. In multiple sclerosis (MS), MEP latency and central delay were often very prolonged. The MEP was more sensitive than the SEP in MS. In amyotrophic lateral sclerosis, MEP latencies were only modestly prolonged; the characteristic abnormality was reduced amplitude. When pseudobulbar features predominated MEPs were often absent. The MEP was of normal latency in Parkinson's disease, but age-related amplitude was often increased. MEP latency and amplitude were normal in Huntington's disease. Abnormal MEPs persisted several months after stroke despite good functional recovery. The MEP could be used to advantage to demonstrate proximal conduction slowing and block in demyelinating neuropathies. In plexopathy, ability to elicit an MEP several days after onset of paresis was good evidence of neuronal continuity in motor fibers.