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Needle EMG Abnormalities in Neurogenic and Muscle Diseases
K. Ming Chan

TYPES OF INTRAMUSCULAR NEEDLE EMG ELECTRODES RATIONALE FOR THE CHOICE OF ELECTRODES NORMAL FINDINGS IN HEALTHY SUBJECTS TYPICAL PATHOLOGICAL NEEDLE EMG FINDINGS IN PATIENTS WITH AXON LOSS NEUROGENIC DISEASES DEMYELINATING NEUROGENIC DISEASES UPPER MOTONEURON DISEASES PRIMARY MUSCLE DISEASES POTENTIAL PITFALLS IN DISTINGUISHING NEUROGENIC FROM MUSCLE DISEASES BASED ON THE NEEDLE EMG FINDINGS Muscle Selection

Adequate Motor Unit Sampling Time Course of the Disease Temperature Choice of Recording Needle Electrodes and Their Site of Insertion Aging Patient Cooperation Fatigue and Other Physiological Factors

The physiological properties of motor units can be affected in many different ways, depending on the underlying disease process. Recognition and an understanding of these patterns of abnormalities can be helpful when one tries to determine the mechanisms of injury and to quantify disease severity. The common abnormal findings in pathological conditions can be broadly divided as those associated with neurogenic versus those associated with myopathic diseases. This approach is useful conceptually in illustrating how motor unit physiological functions are altered, depending on the location and nature of the primary pathology. However, along with this generalization comes the risk of oversimplification. There are often exceptions to these rules and many abnormalities are not unique to either neurogenic or muscle diseases. To avoid these pitfalls, an understanding of the characteristics of the different types of needle electrodes and an appreciation of the range of normality and factors that can affect them are necessary. In this chapter, the following topics are covered: (1) different types of needle electromyographic (EMG) electrodes, (2) rationale for their choice, (3) needle EMG findings in normal individuals to help contrast differences in (4) pathological conditions, and finally (5) potential technical and physiological pitfalls in the interpretation of needle EMG abnormalities.

TYPES OF INTRAMUSCULAR NEEDLE EMG ELECTRODES
The study of many motor unit electrophysiological properties, such as the size of the motor unit

action potential, the firing rate, synchronicity of the electrical conduction, security of electrical transmission through the terminal branches and neuromuscular junction, and excitability of muscle fiber membrane, axon, and motoneuron require the use of microelectrodes that can be placed close to the innervated muscle fibers. Many types of intramuscular needle electrodes have been specifically designed to examine different physiological parameters (Fig. 18–1). To make a sensible choice of the type of electrodes that can best measure the physiological function of interest, a clinician needs to have a good understanding of the specific features associated with each electrode type, their limitations, cost, availability, and potential risk. The commonly used concentric needle electrode, introduced by Adrian and Bronk in the 1920s, has a single insulated wire inside the cannula of a hypodermic needle, fixed in place by epoxy glue and cut flush with the needle tip (Adrian and Bronk, 1929). This recording wire, with a recording surface of 150 by 600 ␮m at the tip, is referenced to the cannula. Another commonly used electrode is a monopolar needle electrode that is made up of an insulated solid needle except at the most distal 300 ␮m at the tip, referenced to a surface electrode; thus, it has a slightly larger pickup area. To study electrical transmission in single muscle fibers, an electrode with a much smaller recording area is required. This electrode, introduced by Stalberg and Ekstedt in the 1960s, with a recording surface of 25 ␮m, is located in a side port 3 mm back from the needle tip on the opposite side of the bevel. (Ekstedt, 1964; Stalberg, 1966). This configu359

has a very large pickup area and can therefore detect the entire motor unit territory. 1. it is crucial that the configuration of the action potentials of the recruited motor units has to remain relatively constant even with small needle displacement. Barkhaus and Nandedkar. the clinician has to choose an electrode that is most appropriate for the task at hand. running the length of a hypodermic needle. macro EMG.2:127. A MEPP is the result of depolarization of the postsynaptic membrane generated by the binding of an acetylcholine vesicle to an acetylcholine receptor. 2. baseline interference. 1980). This recording surface. baseline noise. With increasing concern regarding bloodborne diseases. Regular sharpening and inspection for hooks at the tip are also necessary in order to minimize patient discomfort and damage to the muscle fibers. A monopolar needle electrode that is simply a wire insulated all around except at the tip. an electrode with a pickup territory larger than those described so far is obviously needed. Single-fiber electrode. lying within the pickup area of the single fiber electrode. The insulation coating of the electrode also has to be regularly inspected. For these purposes. disposable needle electrodes are used at an increasingly frequently rate. the small pickup area of the electrode does not provide any information on the electrical size of the whole motor unit. and patient comfort should also be taken into account. The recording surface of the electrode must be kept meticulously clean in order to minimize impedance and. On the other hand. The study of recruitment threshold and firing rate of individual motor units requires reliable identification of their motor unit action potentials. electrical noise. A full description of these electrodes is in the text. while the opposite may be true for others. Therefore. as action potentials generated by individual muscle fibers lying within the very small pickup area of the electrode can be readily discerned and the jitter between the fiber pairs measured. time locked to this spike. At the tip of the needle is a very large recording surface (the area in black measuring 1. The configuration of this electrode is similar to the single fiber electrode except that the distal 1. An added drawback of reusable electrodes is that regular maintenance is required. a macro-EMG needle electrode is needed for deeper muscles (Stalberg. the single-fiber EMG electrode is ideal. 1966. In addition to the aforementioned considerations. These discharges are nonpropagating membrane potentials occurring spontaneously even when the subject is at rest. such as human immunodeficiency virus (HIV) and hepatitis. Four common types of needle EMG electrodes. In normal muscles. as a trigger. 4. CRC Crit Rev Clin Neurobiol 1986. monopolar and concentric needles are reasonable choices. Macro-EMG electrode. are averaged and displayed on the second channel.360 Section IV I Peripheral Motor Sensation Figure 18–1. With motor unit recruitment and subsequent generation of an action potential in the terminal axon. An exception is when the needle is placed at the motor end-plate where irregular discharges will be detected (Fig. Given that the innervation territory of a motor unit can be up to 1 cm in normal individuals and even larger in pathological conditions. Although this information may be obtained with the use of surface electrodes for superficial muscles.) ration helps to minimize the risk of studying muscle fibers damaged by the needle tip during insertion. however. Concentric needle electrode. For studying neuromuscular transmission and . a two-channel setup is required. monophasic high-frequency discharges with a characteristic ‘‘seashell’’ sound.5 cm of the needle electrode is bare. to record from the entire territory of the motor unit. The setup on the right side (4B) is identical to that of the single-fiber electrode.5 cm in length) for detecting the action potentials generated by all the constituent muscle fibers within the motor unit territory (A). The first channel uses the action potential spike generated by a single muscle fiber. the risk of infection. 18–2). 3. small-amplitude. hence. as chipping can also degrade the signal-to-noise ratio. Certain physiological properties are best measured by electrodes with a highly restricted pickup area. NORMAL FINDINGS IN HEALTHY SUBJECTS The first electrical signal one sees on needle EMG examination is insertional activity generated by the muscle fibers when they come into contact with the needle. A full view of the recording surface is shown on the right. The action potentials from all other muscle fibers innervated by the same motoneuron. The recording wire is represented by the stippled area. the frequency and number of the MEPPs will increase. muscle fiber density. The macro-EMG electrode was introduced by Stalberg for this purpose (Stalberg. this only lasts 50 to 150 ms. resulting in spatial and temporal summation to generate an EPP with sufficient amplitude to cause opening of the RATIONALE FOR THE CHOICE OF ELECTRODES Depending on the particular physiological properties of interest. referenced to a surface electrode on the skin. These include miniature end-plate potentials (MEPPs) and end-plate potentials (EPPs). (Adapted by permission from Stalberg E: Single fiber EMG. and scanning EMG: New ways of looking at the motor unit. 1994). Diameter of the uptake area is about 300 ␮m. The MEPPs are irregular.

C. This results in the generation of a propagating action potential that will go on to depolarize the rest of the muscle fiber. In addition to maintaining the force of contraction through modulation of firing rate. a neuropathic motor unit undergoing recent reinnervation has an increased duration. recruitment of additional motor units is another means of increasing force production. where the rising slope of the motor unit action potential is sharper. the motor unit amplitude will gradually increase and the polyphasicity will be reduced. Normal motor units are typically recruited at 6 to 7 Hz. Being generated at the end-plate. a myopathic motor unit is smaller. or changes in the caliber of muscle fibers.318. However. The constituent muscle fibers even in Figure 18–3. Butterworth. (Reprinted by permission from Brown WF: The Physiological and Technical Basis of Electromyography. the examiner usually inserts the needle close to the motor endplate. the steepest incline usually lies between 15 and 30 Hz. positive deflection followed by a larger negative peak and a subsequent slowly recovering positive phase (Fig. Miniature end-plate potentials (MEPPs) can be seen firing at high frequency immediately after needle insertion (A).373. shorter with polyphasicity. the recruitment ratio can be used: dividing the firing frequency of the fastest firing motor unit by the number of different motor units detected by the needle electrode. On the other hand. Although motor units sometimes fire very rapidly at the beginning of a ramp contraction. The end-plate activity could be easily mistaken as abnor- mal spontaneous or insertional activity. they rarely fire at rates higher than 30 Hz once the force has reached a stable plateau during an isometric contraction. Boston. feet. Typical motor unit configurations in myopathic and neuropathic diseases. Although one tries to avoid entering the motor end-plate region because of the associated discomfort. In routine needle EMG examination. the force generated increases in a sigmoidal fashion. In contrast to a normal motor unit (A). particularly in small muscles such as those in the hands. rather than linearly. Recruitment frequency in normal individuals has a range between 6 and 15 Hz. and (2) the MEPPs persist even with the subject completely relaxed but quickly disappear with a small movement of the needle electrode away from the motor endplate. and linked potentials. (Reprinted by permission from Brown WF: The Physiological and Technical Basis of Electromyography. firing semi-irregularly. 18–3A). With increasing firing frequency. number of phases. Boston. 1984. they do not have a preceding positive deflection and are therefore biphasic in configuration. As the motor unit matures. Alternatively. One way of gauging this is by calculating the recruitment frequency—the firing rate of the recruited motor unit when an additional motor unit is recruited.Chapter 18 I Needle EMG Abnormalities in Neurogenic and Muscle Diseases 361 Figure 18–2. Brief trains of EPP may also arise owing to irritation by the advancing needle. Butterworth.) voltage-sensitive Na channels.) . many motor units also have linked potentials—a result of muscle fiber splitting. Depending on the contractile speed of the motor units. Two clues that help the examiner to recognize the motor endplate zone are that (1) the EPPs are small and brief in duration. B. Normal motor unit action potentials are typically triphasic in their configuration: an initial small. it may be encountered inadvertently. The activity died down quickly within one (B) to two minutes (C) when only a few MEPPs are seen. 1984. regeneration of the terminal axons. Recordings obtained by inserting a concentric needle electrode into the end-plate region of a tibialis anterior muscle. or paraspinal muscles.

1982). 1982. Rather. which limits its usefulness. In normal muscles. however. such as amyotrophic lateral sclerosis. Fasciculation is another consequence of peripheral nerve hyperexcitability. the origin of positive sharp waves is much less clear (Dumitru. 1981). 18–5). 1975). However. while positive sharp waves have a large steep initial positivity followed by a slow recovering negative phase (Fig. Albers JW: Prognostic value of ´ syndrome. firing regularly at a much lower frequency. 1965). 11:769– electrodiagnosis in Guillain-Barre 774. A fibrillation potential is also present. 1996). The action potentials on the left panel have a fibrillation potential firing regularly at 12 Hz. Fasciculation potentials discharge irregularly at rates as low as 0.’’ The number and frequency of discharges of individual potentials in the burst and the burst duration and frequency can be quite variable (Albers et al. Muscle Nerve 1988.’’ This hyperexcitability was initially thought to be due to hypersensitivity of muscle fibers to acetylcholine following denervation. the jitter is about 10 to 30 ␮s. TYPICAL PATHOLOGICAL NEEDLE EMG FINDINGS IN PATIENTS WITH AXON LOSS NEUROGENIC DISEASES Denervated muscle fibers become hyperexcitable. Although fasciculation potentials are particularly common and well known in certain diseases.. Clinically. 1988). up to several hertz (Fig. Myokymic discharges characteristically consist of bursts of potentials interspersed with periods of electrical silence. Hyperexcitability of peripheral nerve fibers can also be expressed by other abnormalities. Although simple and easy to use.. Common conditions in which myokymic discharges are found are summarized in Table 18–2. Peterson GW.1 Hz. While there is convincing evidence that fibrillation potentials are extracellularly recorded action potentials generated by single muscle fibers. Both positive sharp waves and fibrillation potentials most commonly discharge regularly with the sound likened to the ‘‘ticking of a clock.362 Section IV I Peripheral Motor Sensation a normal motor unit do not fire completely synchronously. This variability is accounted for by the variance in temporal summation and postsynaptic depolarization and is best appreciated with a single-fiber EMG needle when the ‘‘jitter’’ can be clearly seen. Although some earlier studies suggested that there might be reliable distinguishing features between fasciculation potentials in pathological and normal states. this did not turn out to be the case.’’ The sites at which the fasciculation potentials originate can be anywhere from the dendritic tree to the terminal arbor of the lower motoneuron. . this scale is nonlinear and qualitative. Figure 18–4. leading to partial depolarization and spontaneous oscillation of the membrane potential (Thesleff and Wand. Recording from the tibialis anterior muscle of an 81year-old woman with a peroneal nerve injury using a concentric needle electrode when the patient was at rest. They probably represent ectopic spontaneous discharges generated by injured and compressed nerve fibers. The associated sound of this irregularly discharging pattern has been likened to the sound of ‘‘raindrops on a tin roof. The configuration of the action potentials suggests that they are generated by a part of or an entire motor unit. with a sound likened to that of ‘‘marching soldiers. Fibrillation potentials are small biphasic or triphasic muscle fiber action potentials with brief duration. this did not turn out to be so (Trojaborg and Buchthal. They gradually disappear. they may be due to altered Na channel density and kinetics. A Clinical Scale for Grading the Fibrillation Potentials and Positive Sharp Waves 0 Normal insertional activity 1‫ ם‬Transient but reproducible fibrillation potentials and/or positive sharp waves with needle movements 2‫ ם‬Occasional spontaneous activities in more than two sites 3‫ ם‬Moderate spontaneous activities in all needle sites 4‫ ם‬Abundant spontaneous activities filling the screen From Miller RG. they can also occur in normal individuals. as reinnervation progresses. these changes are commonly represented on a five-point scale (Table 18–1) (Miller et al.. The precise origins of myokymic discharges are unknown. 18–4). Most of the abnormal spontaneous activities on the right panel are positive sharp waves with a regular firing pattern at about 30 Hz. reflected by the presence of fibrillation potentials and positive sharp waves either firing spontaneously or induced by needle movements. Table 18–1. accounting for the variable morphology of the action potentials (Conradi et al. Daube JR. Roth.

18–6). This instability is particularly evident when it is studied with a singlefiber EMG needle. Diseases Associated with Myokymic Discharges Central Nervous System Diseases Multiple sclerosis Pontine tumors Facial nerve palsy Spinal cord injury Peripheral Nervous System Diseases Radiation plexopathy Facial nerve palsy ´ syndrome Guillain-Barre Vasculitic and ischemia neuropathies Figure 18–6. then increased insertional activity. conduction block and a dropout of these components may occur. However. Judging from the differences in their amplitudes. with many spike components and sometimes linked potentials (Fig. as there are fewer motor units contributing to the force production (Fig. 1982). fewer motor units are detected by the recording needle electrode.345.) . Markedly increased jitter and frequency-dependent blocks of the late components can be frequently seen (Stalberg and Trontelj. Boston. Occasionally. The late components are generated by newly formed terminal twigs that are thinly myelinated and therefore can only conduct slowly. there are many fasciculating motor units. newly reinnervated motor units are small. As the caliber of these terminal branches becomes larger and better myelinated. the most notable abnormality in patients with upper motoneuron diseases is a deficit in voluntary recruitment. Motor units. Sumner and colleagues showed that the smaller. (Reprinted by permission from Brown WF: The Physiological and Technical Basis of Electromyography. the interference pattern becomes discrete. If there is additional secondary axon loss. UPPER MOTONEURON DISEASES Characteristically. but the firing rate of the recruitable motor units may be increased. and positive sharp waves will also appear. Consequently. the remaining motor units may have larger-than-expected amplitudes. even Figure 18–5. apart from changes in the recruitment pattern.Chapter 18 I Needle EMG Abnormalities in Neurogenic and Muscle Diseases 363 Table 18–2. Thus. 18–7). the firing rate of the individual motor unit is increased. With the reduced number of motor units and less intermingling of muscle fibers belonging to different motor units in the same area. As a result. 18–3C). the interference pattern becomes discrete and incomplete even with maximal effort. In an axonal injury process. DEMYELINATING NEUROGENIC DISEASES A primary consequence of this is conduction block. As a result. recurrent discharge of the same motor units in the form of doublets or triplets can be seen as a result of ephaptic transmission or recurrent activation of the same motor axons. Fasciculation potentials. lower threshold motor units were more susceptible to conduction block (Sumner et al. This record shows the characteristic highly irregular firing pattern of fasciculation potentials with low firing rates. It shows a hypercomplex motor unit action potential with a very small link potential (*). electrical propagation is sometimes insecure. fibrillation potentials. resulting in fewer available motor units for voluntary recruitment. resulting in disappearance of the polyphasic components and linked potentials—eventually replaced by a triphasic but enlarged motor unit action potential (see Fig. In an experimental demyelination model in rats. electrical conduction will be faster and more secure.. Furthermore. In early stages of reinnervation. configuration of the motor unit action potential is also changed. Butterworth. Recording using a monopolar electrode from the biceps brachii muscle of a 59-year-old man with a very severe axon loss sensorimotor polyneuropathy. especially at high firing frequency. Instability of some of the spike components is evident from the variation in the configuration of the motor unit action potentials. 1984. during the initial weeks and months. 1994).

Although abnormally increased insertional activity is a well-known abnormality in a denervation process or with muscle fiber necrosis. 1951). particularly in the distal limb. These regularly firing action potentials are complex repetitive discharges brought on by needle movements. there are other features. complex repetitive discharges (CRDs) may also be present. The fact that increased insertional activity is also present in muscle diseases. and electrolyte imbalance. This may occur in a number of settings. however. these electrical activities may become spontaneous.364 Section IV I Peripheral Motor Sensation such as multiple sclerosis. Although less appreciated. and positive sharp waves can follow within several weeks after an upper motoneuron lesion in the muscles on the contralateral side. Conversely. myokymic discharges can accompany central nervous system disorders. activated through ephaptic transmission (Fig. The initially activated muscle fiber acts as a pacemaker.. resistance is reduced. This phenomenon is well documented in humans (Goldby.. When the muscle fiber membrane excitability is increased further. Goldkamp. 1983). An important clue to muscle fiber replacement by fibrosis or fatty tissue is that the consistency of the muscle and resistance to the advancing needle are changed. reduced insertional activity is also abnormal. can be explained by segmental muscle fiber necrosis. which effectively results in denervation of the surviving portion of the muscle fibers. depending on the action potentials generated by particular muscle fibers involved in the closed circuit. 18–8) (Trontelj and Stalberg. 1973. when recruited. a lone motor unit was recruited. Three recordings from the tibialis anterior muscle of the same patient in Figure 18–4 while the patient was at rest. initiating the firing at a fairly constant rate until the membrane potential eventually runs down to the point when firing Figure 18–7. 1960). such as polymyositis. other brainstem diseases. In more chronic processes. In the case of fibrotic tissues.. As well. 1990) monkeys (Matthews et al. . PRIMARY MUSCLE DISEASES Increased insertional activity or abnormal spontaneous activity may be present if there has been a substantial amount of muscle fiber necrosis. such as profound hypokalemia or during periodic paralysis. Configurations of the action potentials in the three panels are markedly different. 1957. Brown and Snow. The complex configuration of CRDs is explained by the fact that they consist of action potentials generated by individual muscle fibers forming a closed circuit. McComas et al. A monopolar needle recording from the tibialis anterior muscle of a 60-year-old man who sustained a severe sciatic nerve injury after a hip dislocation 2 years earlier with the use of a monopolar electrode. 1967. fatty infiltrates. In addition to this. Figure 18–8. and other mammalian species (Cook et al. can fire only slowly and in a poorly sustained manner. the muscle feels rubbery and its resistance to needle insertion is increased. firing at frequencies between 15 and 25 Hz without any sign of other additional motor unit recruitment. spontaneous discharge of fibrillation potentials. and spinal cord injury. increased insertional activity. including muscle fibrosis. The underlying mechanism is thought to be due to ‘‘transsynaptic degeneration.’’ when the loss of input from the upper motoneurons induces death of the lower motoneurons. activated and sustained through ephaptic transmission. In this record. in the case of fatty infiltrate.

18–9). muscle fibers may be abnormally excitable. positive sharp waves. clinical information is crucial in guiding the proper interpretation of the EMG findings. The more commonly associated conditions are listed in Table 18–3. the motor unit amplitude is reduced. 657. 1953). this symptom is particularly troublesome in myotonia congenita. For example. The end result is that the resting membrane potential becomes unstable. the duration of the motor unit action potential. at times partially depolarized and hence hyperexcitable (Iaizzo. particularly in Duchenne’s muscular dystrophy stops abruptly. Therefore. Patients with paramyotonia are particularly sensitive to cold when severe muscle contracture can develop. in both amplitude and frequency. giving rise to the classic ‘‘motorcycle’’ or ‘‘dive-bomber’’ sound. The interference pattern is usually full very early even at very low levels of contraction because of the limited force-generating capacity of the affected muscle fibers. many abnormalities are not unique to either entity. 18–10). However. Although similar in some ways. myotonic discharges have a cyclical ‘‘waxing and waning’’ firing pattern with characteristic sounds likened to those coming from a revving motorcycle or a ‘‘divebomber’’ (Fig. sometimes at rates as high as several hundred hertz (Fig. The opposite may be seen in severely affected muscles in muscle diseases when secondary axonal degeneration can occur. However. hypercomplex motor unit action potentials. 1991). The same is true for small amplitude. Clinically. The morphological appearance of the action potentials. This fact could explain the high incidence of late components found in patients with Duchenne muscular dystrophy (Desmedt and Borenstein. in turn. (Reprinted by permission from Kimura J: Electrodiagnosis in Diseases of Nerve and Muscle: Principles and Practice. Copyright 1989 by Oxford University Press. In contrast. 1993). neuromyotonia should not be confused with myotonia. their conduction velocities also become increasingly varied which. 2nd ed. 1993). brief biphasic or sometimes monophasic spikes. fibrillation potentials. In addition. the amplitude may progressively diminish as the disease progresses when the terminal branches begin to die off and muscle atrophy ensues. Myotonic discharges with a characteristic ‘‘waxing and waning’’ pattern. 1949. The motor unit action potential configuration in primary muscle diseases is also altered. Inc. Even though large-amplitude motor unit action potentials are classically associated with axon loss neuropathies. Buchthal and Pinelli. giving rise to large motor unit action potentials. 1976).Chapter 18 I Needle EMG Abnormalities in Neurogenic and Muscle Diseases 365 Table 18–3.) Muscle Selection Different muscles are often preferentially affected in different diseases. Diseases Associated with Complex Repetitive Discharges Neurogenic Diseases Spinal muscular atrophy Charcot-Marie-Tooth disease Amyotrophic lateral sclerosis Radiculopathy Chronic polyneuropathies Primary Muscle Diseases Inflammatory myositis Muscular dystrophies. Classically. the muscle stiffness tends to improve when the muscle warms up. this is not always observed in recordings with concentric and monopolar electrodes as the motor unit action potential amplitude is highly influenced by the muscle fibers located immediately adjacent to the recording surface of these electrodes. on needle examination. Myotonic discharges can have a number of underlying mechanisms including dysfunctional changes affecting the ion channels (Ptacek et al. This is associated with rare conditions such as Isaacs’ syndrome or stiff-man syndrome (Newsom-Davis and Mills. To optimize the sensitivity of . has been found to be a more reliable parameter (Kugelberg. suggests that they are probably generated by single muscle fibers. would lengthen the duration. more so than in myotonic dystrophy. an observation against this is that as the affected muscle fibers undergo varying degrees of atrophy. New York.. and CRDs can be seen in both. In myotonic disorders. Oxford University Press. The reduced duration is thought to be the result of muscle fiber loss resulting in less temporal dispersion. POTENTIAL PITFALLS IN DISTINGUISHING NEUROGENIC FROM MUSCLE DISEASES BASED ON THE NEEDLE EMG FINDINGS Although there are many differences in needle EMG examination findings that can help to distinguish neurogenic disorders from muscle diseases. resulting in muscle stiffness. As the result of muscle fiber atrophy. However. which is typically reduced in muscle diseases. CRDs are present not only in primary muscle diseases but also in many chronic axon loss neurogenic disorders. a number of potential confounding factors may further blur the distinction between the two entities: Figure 18–9. Neuromyotonic discharges can have a wide variety of firing patterns.

interpretation based on isolated findings of one or two large or polyphasic motor unit action potentials can be potentially misleading. appropriate muscle selection is crucial. one feature that differentiated it from stiff-man syndrome. an adequate number of sites in the muscle must be studied as the affected areas may be widely scattered. Neuromyotonic discharges were present in numerous muscles on her arms. of which the aforementioned four muscles are just a few examples. The spontaneous firing did not disappear even when the patient was sedated and during sleep. rather than the primary disease of interest. in a cervical radiculopathy. Conversely. these abnormalities eventually disappear as the denervated muscle fibers Adequate Motor Unit Sampling As well. adequate sampling is crucial to avoiding misinterpretation. as is often the case in mild polymyositis. In early stages of a nerve injury. malities evolve over time. Distal muscles innervated by nerves.3:429. depending on the distance between the site of injury and the muscle studied. and face. For example. Since the recording surface of most needle EMG electrodes is highly restricted. Over time. the number of motor units that can be sampled at any one site is limited. fibrillation potentials and positive sharp waves could take up to several weeks to develop. Neurology 1953. (Adapted by permission from Buchthal F. Examination of moderately weak muscles is often more helpful than looking at muscles that are already markedly affected. it may take up to 3 weeks before these changes are seen in the distal forearm and hand muscles. following an acute axon loss injury. Pinelli P: Muscle action potentials in polymyositis. This limitation is further compounded by the facts that the physiological properties of the constituent motor units in a muscle usually span a wide range and that there is often a considerable overlap in their distribution between normal and disease (Fig.) . 18–11). legs. that are prone to focal compression may add confounding features related to the compression. such as the median or ulnar nerves. Time Course of the Disease Knowledge of this and the rate of progression are also important as most electrophysiological abnor- Figure 18–11. This further emphasizes the need for adequate sampling at different sites as a great necessity.366 Section IV I Peripheral Motor Sensation Figure 18–10. Monopolar needle recordings from a 69-year-old woman with Isaacs’ syndrome while the patient was at rest. For example. trunk. the needle EMG examination. The data are superimposed to illustrate the substantial overlap between the two groups. as the pathological features can become murky in advanced disease. The discharges are mostly of such high frequencies that it is impossible to discern the configuration of the individual action potentials. such as a radiculopathy. most myopathies affect proximal muscles more severely where the examination should be directed. Therefore. The distributions of motor unit action potential duration in patients with polymyositis compared with normal subjects. For example. in which the pathological changes may be found only in a few areas in some of the innervated muscles. the fibrillation potentials and positive sharp waves first appear in muscles immediately downstream from the site of injury and later in the more distal muscles. The same also applies to neurogenic diseases.

Miller et al. However. Choice of Recording Needle Electrodes and Their Site of Insertion Another important physical factor is the relative size of the pickup area of the electrode used in comparison to the size of the muscle. presumably as the result of increased phase cancellation (Buchthal et al. Patient Cooperation The ability to volitionally recruit motor units is obviously influenced by the subject’s cooperation. Doherty et al. inability to follow commands.. Finally. Muscle Nerve 1981. hypercomplex motor unit action potentials. It also increases the time constant of Na and K channel opening. 1987). the needle examination is still potentially useful as the motor unit recruitment frequency and recruitment ratio are not influenced by the previously mentioned volitional factors. Hyperventilation and limb ischemia could lead to fasciculation potentials in otherwise healthy individuals. the amplitude measured by a macro-EMG needle electrode more accurately reflects the overall size of the motor unit. Brown WF: The Physiological and Technical Basis of Electromyography. In the elderly. Temperature Temperature can have a profound influence on neuromuscular transmission and propagation of the action potential along the muscle fibers. Part II. . or pain. duration of the motor unit action potential is longer when recorded with concentric and monopolar electrodes. 1996. J Physiol 1929. Daube JR: Limb myokymia. Griffin et al. the amplitude and duration of the motor unit action potentials are increased. Dorfman et al. no abnormal insertional or spontaneous activity is detected in the healthy elderly. Such examples include the intrinsic hand muscles and the extensor digitorum brevis muscle in the foot. Spontaneous activity. such as when a macro-EMG needle with a large pickup area is used to record from a small intrinsic or foot muscle. if there is ongoing denervation... As a result.Chapter 18 I Needle EMG Abnormalities in Neurogenic and Muscle Diseases 367 become reinnervated and replaced by small. Cold temperature increases the safety margin of neuromuscular junction transmission by reducing the release of acetylcholine vesicles from the presynaptic terminal. However. Bigland-Ritchie et al. Therefore. Fatigue can induce changes in the configuration of the motor unit action potentials and alter their recruitment pattern and firing rate (Maton. compared with bipolar electrodes. sometimes required when one attempts to record a large number of motor unit action potentials for quantitative analysis. a larger proportion of enlarged motor units may be present. 1998). the amplitude of a motor unit action potential recorded by concentric or monopolar needle electrode is highly influenced by the muscle fibers in the immediate vicinity of the recording surface. Poor recruitment could be the result of a subject’s unwillingness to cooperate. as is often the case in many muscle diseases. and increasing the sensitivity of the acetylcholine receptors on the postsynaptic membrane. 1993. 1973. Aging Motor unit loss associated with aging is well known (Campbell et al. 1990. resulting in polyphasicity. Bigland-Ritchie B. 1995). the rate and extent of loss may be more rapid and more marked in muscles that are prone to trauma or innervated by nerves vulnerable to compression. The site of needle insertion in relation to the motor endplate and the angle of insertion in relation to the muscle fiber plane can also affect the configuration and duration of the motor unit action potential. Cafarelli E. Finally. In these muscles. Christova and Kossev.. Larsson and Ansved. Barkhaus PE. the number and frequency of MEPPs are reduced.. 1954). A major mismatch. References Adrian ED.4:494–504.. Nandedkar SD: Recording characteristics of the surface EMG electrodes. resulting in a marked lengthening of the muscle fiber action potential duration. despite these difficulties. as the result of chronic reinnervation. Fatigue and Other Physiological Factors Muscle fatigue may occur after prolonged contractions.. 1986. misunderstanding of the required task. a reflection of membrane stability.67:119–151. Bastron JA. the number of spikes in a motor unit action potential increases.17:1317–1323. and the jitter is increased. As a result of conduction slowing. Bronk DW: The discharge of impulses in motor nerve fibres.556:137– 148. This is particularly likely to occur in conditions in which the fiber density is increased. The frequency of discharge in reflex and voluntary contractions. the positive sharp waves and fibrillation potentials may continue to persist indefinitely (Cashman et al. Vollestad NK: Fatigue of submaximal static contractions. could result in a very noisy baseline from the firing of neighboring muscles. 1984. The amplitude falls moderately with cooling. Boston. is reduced with cooling. Since the rate of loss is usually very slow.. decreasing the rate of degradation of the acetylcholine molecules in the synaptic cleft. Butterworth. Muscle Nerve 1994. However. Albers JW. 1998. Acta Physiol Scand Suppl 1986. Allen AA 2d. 1981.

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