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18

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 ndings 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 oversimplication. 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 ndings in normal individuals to help contrast differences in (4) pathological conditions, and nally (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 ring rate, synchronicity of the electrical conduction, security of electrical transmission through the terminal branches and neuromuscular junction, and excitability of muscle ber membrane, axon, and motoneuron require the use of microelectrodes that can be placed close to the innervated muscle bers. Many types of intramuscular needle electrodes have been specically designed to examine different physiological parameters (Fig. 181). 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 specic 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, xed in place by epoxy glue and cut ush 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 bers, 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 congu359

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Figure 181. Four common types of needle EMG electrodes. 1, Concentric needle electrode. The recording wire is represented by the stippled area, running the length of a hypodermic needle. A full view of the recording surface is shown on the right. 2, A monopolar needle electrode that is simply a wire insulated all around except at the tip. 3, Single-ber electrode. 4, Macro-EMG electrode. The setup on the right side (4B) is identical to that of the single-ber electrode. At the tip of the needle is a very large recording surface (the area in black measuring 1.5 cm in length) for detecting the action potentials generated by all the constituent muscle bers within the motor unit territory (A). A full description of these electrodes is in the text. (Adapted by permission from Stalberg E: Single ber EMG, macro EMG, and scanning EMG: New ways of looking at the motor unit. CRC Crit Rev Clin Neurobiol 1986;2:127.)

ration helps to minimize the risk of studying muscle bers damaged by the needle tip during insertion. Diameter of the uptake area is about 300 m. Given that the innervation territory of a motor unit can be up to 1 cm in normal individuals and even larger in pathological conditions, an electrode with a pickup territory larger than those described so far is obviously needed. The macro-EMG electrode was introduced by Stalberg for this purpose (Stalberg, 1980). The conguration of this electrode is similar to the single ber electrode except that the distal 1.5 cm of the needle electrode is bare. This recording surface, referenced to a surface electrode on the skin, has a very large pickup area and can therefore detect the entire motor unit territory; however, to record from the entire territory of the motor unit, a two-channel setup is required. The rst channel uses the action potential spike generated by a single muscle ber, lying within the pickup area of the single ber electrode, as a trigger. The action potentials from all other muscle bers innervated by the same motoneuron, time locked to this spike, are averaged and displayed on the second channel.

muscle ber density, the single-ber EMG electrode is ideal, as action potentials generated by individual muscle bers lying within the very small pickup area of the electrode can be readily discerned and the jitter between the ber pairs measured. On the other hand, the small pickup area of the electrode does not provide any information on the electrical size of the whole motor unit. Although this information may be obtained with the use of surface electrodes for supercial muscles, a macro-EMG needle electrode is needed for deeper muscles (Stalberg, 1966; Barkhaus and Nandedkar, 1994). The study of recruitment threshold and ring rate of individual motor units requires reliable identication of their motor unit action potentials. Therefore, it is crucial that the conguration of the action potentials of the recruited motor units has to remain relatively constant even with small needle displacement. For these purposes, monopolar and concentric needles are reasonable choices. In addition to the aforementioned considerations, the risk of infection, baseline interference, electrical noise, and patient comfort should also be taken into account. With increasing concern regarding bloodborne diseases, such as human immunodeciency virus (HIV) and hepatitis, disposable needle electrodes are used at an increasingly frequently rate. An added drawback of reusable electrodes is that regular maintenance is required. The recording surface of the electrode must be kept meticulously clean in order to minimize impedance and, hence, baseline noise. The insulation coating of the electrode also has to be regularly inspected, as chipping can also degrade the signal-to-noise ratio. Regular sharpening and inspection for hooks at the tip are also necessary in order to minimize patient discomfort and damage to the muscle bers.

NORMAL FINDINGS IN HEALTHY SUBJECTS


The rst electrical signal one sees on needle EMG examination is insertional activity generated by the muscle bers when they come into contact with the needle. In normal muscles, this only lasts 50 to 150 ms. An exception is when the needle is placed at the motor end-plate where irregular discharges will be detected (Fig. 182). These include miniature end-plate potentials (MEPPs) and end-plate potentials (EPPs). A MEPP is the result of depolarization of the postsynaptic membrane generated by the binding of an acetylcholine vesicle to an acetylcholine receptor. The MEPPs are irregular, small-amplitude, monophasic high-frequency discharges with a characteristic seashell sound. These discharges are nonpropagating membrane potentials occurring spontaneously even when the subject is at rest. With motor unit recruitment and subsequent generation of an action potential in the terminal axon, the frequency and number of the MEPPs will increase, resulting in spatial and temporal summation to generate an EPP with sufcient amplitude to cause opening of the

RATIONALE FOR THE CHOICE OF ELECTRODES


Depending on the particular physiological properties of interest, the clinician has to choose an electrode that is most appropriate for the task at hand. Certain physiological properties are best measured by electrodes with a highly restricted pickup area, while the opposite may be true for others. For studying neuromuscular transmission and

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Figure 182. Recordings obtained by inserting a concentric needle electrode into the end-plate region of a tibialis anterior muscle. Miniature end-plate potentials (MEPPs) can be seen ring at high frequency immediately after needle insertion (A). The activity died down quickly within one (B) to two minutes (C) when only a few MEPPs are seen. (Reprinted by permission from Brown WF: The Physiological and Technical Basis of Electromyography. Boston, Butterworth, 1984;373.)

voltage-sensitive Na channels. This results in the generation of a propagating action potential that will go on to depolarize the rest of the muscle ber. Brief trains of EPP may also arise owing to irritation by the advancing needle. In routine needle EMG examination, the examiner usually inserts the needle close to the motor endplate, where the rising slope of the motor unit action potential is sharper. Although one tries to avoid entering the motor end-plate region because of the associated discomfort, it may be encountered inadvertently, particularly in small muscles such as those in the hands, feet, or paraspinal muscles. The end-plate activity could be easily mistaken as abnor-

mal spontaneous or insertional activity. Two clues that help the examiner to recognize the motor endplate zone are that (1) the EPPs are small and brief in duration. Being generated at the end-plate, they do not have a preceding positive deection and are therefore biphasic in conguration; 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. Normal motor units are typically recruited at 6 to 7 Hz, ring semi-irregularly. With increasing ring frequency, the force generated increases in a sigmoidal fashion, rather than linearly. Depending on the contractile speed of the motor units, the steepest incline usually lies between 15 and 30 Hz. Although motor units sometimes re very rapidly at the beginning of a ramp contraction, they rarely re at rates higher than 30 Hz once the force has reached a stable plateau during an isometric contraction. In addition to maintaining the force of contraction through modulation of ring rate, recruitment of additional motor units is another means of increasing force production. One way of gauging this is by calculating the recruitment frequencythe ring rate of the recruited motor unit when an additional motor unit is recruited. Recruitment frequency in normal individuals has a range between 6 and 15 Hz. Alternatively, the recruitment ratio can be used: dividing the ring frequency of the fastest ring motor unit by the number of different motor units detected by the needle electrode. Normal motor unit action potentials are typically triphasic in their conguration: an initial small, positive deection followed by a larger negative peak and a subsequent slowly recovering positive phase (Fig. 183A). The constituent muscle bers even in

Figure 183. Typical motor unit congurations in myopathic and neuropathic diseases. B, In contrast to a normal motor unit (A), a myopathic motor unit is smaller, shorter with polyphasicity. However, many motor units also have linked potentialsa result of muscle ber splitting, regeneration of the terminal axons, or changes in the caliber of muscle bers. C, On the other hand, a neuropathic motor unit undergoing recent reinnervation has an increased duration, number of phases, and linked potentials. As the motor unit matures, the motor unit amplitude will gradually increase and the polyphasicity will be reduced. (Reprinted by permission from Brown WF: The Physiological and Technical Basis of Electromyography. Boston, Butterworth, 1984;318.)

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a normal motor unit do not re completely synchronously. This variability is accounted for by the variance in temporal summation and postsynaptic depolarization and is best appreciated with a single-ber EMG needle when the jitter can be clearly seen. In normal muscles, the jitter is about 10 to 30 s.

Table 181. A Clinical Scale for Grading the Fibrillation Potentials and Positive Sharp Waves
0 Normal insertional activity 1 Transient but reproducible brillation potentials and/or positive sharp waves with needle movements 2 Occasional spontaneous activities in more than two sites 3 Moderate spontaneous activities in all needle sites 4 Abundant spontaneous activities lling the screen
From Miller RG, Peterson GW, Daube JR, Albers JW: Prognostic value of syndrome. Muscle Nerve 1988; 11:769 electrodiagnosis in Guillain-Barre 774.

TYPICAL PATHOLOGICAL NEEDLE EMG FINDINGS IN PATIENTS WITH AXON LOSS NEUROGENIC DISEASES
Denervated muscle bers become hyperexcitable, reected by the presence of brillation potentials and positive sharp waves either ring spontaneously or induced by needle movements. Fibrillation potentials are small biphasic or triphasic muscle ber action potentials with brief duration, while positive sharp waves have a large steep initial positivity followed by a slow recovering negative phase (Fig. 184). While there is convincing evidence that brillation potentials are extracellularly recorded action potentials generated by single muscle bers, the origin of positive sharp waves is much less clear (Dumitru, 1996). Both positive sharp waves and brillation potentials most commonly discharge regularly with the sound likened to the ticking of a clock. This hyperexcitability was initially thought to be due to hypersensitivity of muscle bers to acetylcholine following denervation. However, this did not turn out to be the case. Rather, they may be due to altered Na channel density and kinetics, leading to partial depolarization and spontaneous oscillation of the membrane potential (Thesleff and Wand, 1975). They gradually disappear, however, as reinnervation progresses. Clinically, these changes are commonly represented on a ve-point scale (Table 181) (Miller et al., 1988). Although simple and easy to use, this scale is nonlinear and qualitative, which limits its usefulness. Hyperexcitability of peripheral nerve bers can also be expressed by other abnormalities. Myokymic discharges characteristically consist of bursts of potentials interspersed

with periods of electrical silence, with a sound likened to that of marching soldiers. 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., 1981). The conguration of the action potentials suggests that they are generated by a part of or an entire motor unit. The precise origins of myokymic discharges are unknown. They probably represent ectopic spontaneous discharges generated by injured and compressed nerve bers. Common conditions in which myokymic discharges are found are summarized in Table 182. Fasciculation is another consequence of peripheral nerve hyperexcitability. Fasciculation potentials discharge irregularly at rates as low as 0.1 Hz, up to several hertz (Fig. 185). The associated sound of this irregularly discharging pattern has been likened to the sound of raindrops on a tin roof. The sites at which the fasciculation potentials originate can be anywhere from the dendritic tree to the terminal arbor of the lower motoneuron, accounting for the variable morphology of the action potentials (Conradi et al., 1982; Roth, 1982). Although fasciculation potentials are particularly common and well known in certain diseases, such as amyotrophic lateral sclerosis, they can also occur in normal individuals. 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 so (Trojaborg and Buchthal, 1965).

Figure 184. 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. Most of the abnormal spontaneous activities on the right panel are positive sharp waves with a regular ring pattern at about 30 Hz. A brillation potential is also present, ring regularly at a much lower frequency. The action potentials on the left panel have a brillation potential ring regularly at 12 Hz.

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Needle EMG Abnormalities in Neurogenic and Muscle Diseases 363

Table 182. 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 186. Recording using a monopolar electrode from the biceps brachii muscle of a 59-year-old man with a very severe axon loss sensorimotor polyneuropathy. It shows a hypercomplex motor unit action potential with a very small link potential (*). Instability of some of the spike components is evident from the variation in the conguration of the motor unit action potentials.

In an axonal injury process, apart from changes in the recruitment pattern, conguration of the motor unit action potential is also changed. In early stages of reinnervation, during the initial weeks and months, newly reinnervated motor units are small, with many spike components and sometimes linked potentials (Fig. 186). The late components are generated by newly formed terminal twigs that are thinly myelinated and therefore can only conduct slowly. Furthermore, electrical propagation is sometimes insecure, especially at high ring frequency. Consequently, conduction block and a dropout of these components may occur. This instability is particularly evident when it is studied with a singleber EMG needle. Markedly increased jitter and frequency-dependent blocks of the late components can be frequently seen (Stalberg and Trontelj, 1994). As the caliber of these terminal branches becomes larger and better myelinated, electrical conduction will be faster and more secure, resulting in disappearance of the polyphasic components and linked potentialseventually replaced by a triphasic but enlarged motor unit action potential (see Fig. 183C). With the reduced number of motor units and less intermingling of muscle bers belonging to different motor units in the same area, fewer motor units are detected by the recording needle electrode. As a result, the interference pattern becomes discrete and incomplete even with maximal effort. However, the ring rate of the individual motor unit is increased, as there are fewer motor units contributing to the force production (Fig. 187).

DEMYELINATING NEUROGENIC DISEASES


A primary consequence of this is conduction block, resulting in fewer available motor units for voluntary recruitment. Thus, the interference pattern becomes discrete, but the ring rate of the recruitable motor units may be increased. Occasionally, 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. If there is additional secondary axon loss, then increased insertional activity, brillation potentials, and positive sharp waves will also appear. In an experimental demyelination model in rats, Sumner and colleagues showed that the smaller, lower threshold motor units were more susceptible to conduction block (Sumner et al., 1982). As a result, the remaining motor units may have larger-than-expected amplitudes.

UPPER MOTONEURON DISEASES


Characteristically, the most notable abnormality in patients with upper motoneuron diseases is a decit in voluntary recruitment. Motor units, even

Figure 185. Fasciculation potentials. This record shows the characteristic highly irregular ring pattern of fasciculation potentials with low ring rates. Judging from the differences in their amplitudes, there are many fasciculating motor units. (Reprinted by permission from Brown WF: The Physiological and Technical Basis of Electromyography. Boston, Butterworth, 1984;345.)

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such as multiple sclerosis, other brainstem diseases, and spinal cord injury.

PRIMARY MUSCLE DISEASES


Increased insertional activity or abnormal spontaneous activity may be present if there has been a substantial amount of muscle ber necrosis. The fact that increased insertional activity is also present in muscle diseases, such as polymyositis, can be explained by segmental muscle ber necrosis, which effectively results in denervation of the surviving portion of the muscle bers. When the muscle ber membrane excitability is increased further, these electrical activities may become spontaneous. Although abnormally increased insertional activity is a well-known abnormality in a denervation process or with muscle ber necrosis, reduced insertional activity is also abnormal. This may occur in a number of settings, including muscle brosis, fatty inltrates, and electrolyte imbalance, such as profound hypokalemia or during periodic paralysis. An important clue to muscle ber replacement by brosis or fatty tissue is that the consistency of the muscle and resistance to the advancing needle are changed. In the case of brotic tissues, the muscle feels rubbery and its resistance to needle insertion is increased. Conversely, in the case of fatty inltrate, resistance is reduced. In more chronic processes, complex repetitive discharges (CRDs) may also be present. The complex conguration of CRDs is explained by the fact that they consist of action potentials generated by individual muscle bers forming a closed circuit, activated through ephaptic transmission (Fig. 188) (Trontelj and Stalberg, 1983). The initially activated muscle ber acts as a pacemaker, initiating the ring at a fairly constant rate until the membrane potential eventually runs down to the point when ring

Figure 187. 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. In this record, a lone motor unit was recruited, ring at frequencies between 15 and 25 Hz without any sign of other additional motor unit recruitment.

when recruited, can re only slowly and in a poorly sustained manner. In addition to this, however, there are other features. Although less appreciated, increased insertional activity, spontaneous discharge of brillation potentials, and positive sharp waves can follow within several weeks after an upper motoneuron lesion in the muscles on the contralateral side, particularly in the distal limb. 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. This phenomenon is well documented in humans (Goldby, 1957; Goldkamp, 1967; McComas et al., 1973; Brown and Snow, 1990) monkeys (Matthews et al., 1960), and other mammalian species (Cook et al., 1951). As well, myokymic discharges can accompany central nervous system disorders,

Figure 188. Three recordings from the tibialis anterior muscle of the same patient in Figure 184 while the patient was at rest. These regularly ring action potentials are complex repetitive discharges brought on by needle movements. Congurations of the action potentials in the three panels are markedly different, depending on the action potentials generated by particular muscle bers involved in the closed circuit, activated and sustained through ephaptic transmission.

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Needle EMG Abnormalities in Neurogenic and Muscle Diseases 365

Table 183. Diseases Associated with Complex Repetitive Discharges


Neurogenic Diseases Spinal muscular atrophy Charcot-Marie-Tooth disease Amyotrophic lateral sclerosis Radiculopathy Chronic polyneuropathies Primary Muscle Diseases Inammatory myositis Muscular dystrophies, particularly in Duchennes muscular dystrophy

stops abruptly. CRDs are present not only in primary muscle diseases but also in many chronic axon loss neurogenic disorders. The more commonly associated conditions are listed in Table 183. In myotonic disorders, muscle bers may be abnormally excitable, resulting in muscle stiffness. Classically, on needle examination, myotonic discharges have a cyclical waxing and waning ring pattern with characteristic sounds likened to those coming from a revving motorcycle or a divebomber (Fig. 189). The morphological appearance of the action potentials, brief biphasic or sometimes monophasic spikes, suggests that they are probably generated by single muscle bers. Clinically, this symptom is particularly troublesome in myotonia congenita, more so than in myotonic dystrophy. However, the muscle stiffness tends to improve when the muscle warms up. Patients with paramyotonia are particularly sensitive to cold when severe muscle contracture can develop. Myotonic discharges can have a number of underlying mechanisms including dysfunctional changes affecting the ion channels (Ptacek et al., 1993). The end result is that the resting membrane potential becomes unstable, at times partially depolarized and hence hyperexcitable (Iaizzo, 1991). Although similar in some ways, neuromyotonia should not be confused with myotonia. Neuromyotonic discharges can have a wide variety of ring patterns, sometimes at rates as high as several hundred hertz (Fig. 1810). This is associated with rare conditions such as Isaacs

syndrome or stiff-man syndrome (Newsom-Davis and Mills, 1993). The motor unit action potential conguration in primary muscle diseases is also altered. As the result of muscle ber atrophy, the motor unit amplitude is reduced. However, this is not always observed in recordings with concentric and monopolar electrodes as the motor unit action potential amplitude is highly inuenced by the muscle bers located immediately adjacent to the recording surface of these electrodes. In contrast, the duration of the motor unit action potential, which is typically reduced in muscle diseases, has been found to be a more reliable parameter (Kugelberg, 1949; Buchthal and Pinelli, 1953). The reduced duration is thought to be the result of muscle ber loss resulting in less temporal dispersion. However, an observation against this is that as the affected muscle bers undergo varying degrees of atrophy, their conduction velocities also become increasingly varied which, in turn, would lengthen the duration. This fact could explain the high incidence of late components found in patients with Duchenne muscular dystrophy (Desmedt and Borenstein, 1976). 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 bers.

POTENTIAL PITFALLS IN DISTINGUISHING NEUROGENIC FROM MUSCLE DISEASES BASED ON THE NEEDLE EMG FINDINGS
Although there are many differences in needle EMG examination ndings that can help to distinguish neurogenic disorders from muscle diseases, many abnormalities are not unique to either entity. For example, brillation potentials, positive sharp waves, and CRDs can be seen in both. The same is true for small amplitude, hypercomplex motor unit action potentials. Even though large-amplitude motor unit action potentials are classically associated with axon loss neuropathies, the amplitude may progressively diminish as the disease progresses when the terminal branches begin to die off and muscle atrophy ensues. The opposite may be seen in severely affected muscles in muscle diseases when secondary axonal degeneration can occur, giving rise to large motor unit action potentials. Therefore, clinical information is crucial in guiding the proper interpretation of the EMG ndings. In addition, a number of potential confounding factors may further blur the distinction between the two entities:

Figure 189. Myotonic discharges with a characteristic waxing and waning pattern, in both amplitude and frequency, giving rise to the classic motorcycle or dive-bomber sound. (Reprinted by permission from Kimura J: Electrodiagnosis in Diseases of Nerve and Muscle: Principles and Practice, 2nd ed. New York, Oxford University Press, 657. Copyright 1989 by Oxford University Press, Inc.)

Muscle Selection
Different muscles are often preferentially affected in different diseases. To optimize the sensitivity of

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Figure 1810. Monopolar needle recordings from a 69-year-old woman with Isaacs syndrome while the patient was at rest. Neuromyotonic discharges were present in numerous muscles on her arms, legs, trunk, and face, of which the aforementioned four muscles are just a few examples. The spontaneous ring did not disappear even when the patient was sedated and during sleep, one feature that differentiated it from stiff-man syndrome. The discharges are mostly of such high frequencies that it is impossible to discern the conguration of the individual action potentials.

the needle EMG examination, appropriate muscle selection is crucial. For example, most myopathies affect proximal muscles more severely where the examination should be directed. Examination of moderately weak muscles is often more helpful than looking at muscles that are already markedly affected, as the pathological features can become murky in advanced disease. Distal muscles innervated by nerves, such as the median or ulnar nerves, that are prone to focal compression may add confounding features related to the compression, rather than the primary disease of interest.

malities evolve over time. For example, following an acute axon loss injury, brillation potentials and positive sharp waves could take up to several weeks to develop, depending on the distance between the site of injury and the muscle studied. In early stages of a nerve injury, the brillation potentials and positive sharp waves rst appear in muscles immediately downstream from the site of injury and later in the more distal muscles. For example, in a cervical radiculopathy, it may take up to 3 weeks before these changes are seen in the distal forearm and hand muscles. Over time, these abnormalities eventually disappear as the denervated muscle bers

Adequate Motor Unit Sampling


As well, an adequate number of sites in the muscle must be studied as the affected areas may be widely scattered, as is often the case in mild polymyositis. The same also applies to neurogenic diseases, such as a radiculopathy, in which the pathological changes may be found only in a few areas in some of the innervated muscles. Since the recording surface of most needle EMG electrodes is highly restricted, the number of motor units that can be sampled at any one site is limited. 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. 1811). This further emphasizes the need for adequate sampling at different sites as a great necessity. Conversely, interpretation based on isolated ndings of one or two large or polyphasic motor unit action potentials can be potentially misleading.

Time Course of the Disease


Knowledge of this and the rate of progression are also important as most electrophysiological abnor-

Figure 1811. The distributions of motor unit action potential duration in patients with polymyositis compared with normal subjects. The data are superimposed to illustrate the substantial overlap between the two groups. Therefore, adequate sampling is crucial to avoiding misinterpretation. (Adapted by permission from Buchthal F, Pinelli P: Muscle action potentials in polymyositis. Neurology 1953;3:429.)

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become reinnervated and replaced by small, hypercomplex motor unit action potentials. However, if there is ongoing denervation, the positive sharp waves and brillation potentials may continue to persist indenitely (Cashman et al., 1987).

Aging
Motor unit loss associated with aging is well known (Campbell et al., 1973; Doherty et al., 1993; Larsson and Ansved, 1995). Since the rate of loss is usually very slow, no abnormal insertional or spontaneous activity is detected in the healthy elderly. However, as the result of chronic reinnervation, the amplitude and duration of the motor unit action potentials are increased. In the elderly, 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. Such examples include the intrinsic hand muscles and the extensor digitorum brevis muscle in the foot. In these muscles, a larger proportion of enlarged motor units may be present.

Temperature
Temperature can have a profound inuence on neuromuscular transmission and propagation of the action potential along the muscle bers. Cold temperature increases the safety margin of neuromuscular junction transmission by reducing the release of acetylcholine vesicles from the presynaptic terminal, decreasing the rate of degradation of the acetylcholine molecules in the synaptic cleft, and increasing the sensitivity of the acetylcholine receptors on the postsynaptic membrane. As a result, the number and frequency of MEPPs are reduced, and the jitter is increased. It also increases the time constant of Na and K channel opening, resulting in a marked lengthening of the muscle ber action potential duration. As a result of conduction slowing, the number of spikes in a motor unit action potential increases, resulting in polyphasicity. The amplitude falls moderately with cooling, presumably as the result of increased phase cancellation (Buchthal et al., 1954). Spontaneous activity, a reection of membrane stability, is reduced with cooling.

Patient Cooperation
The ability to volitionally recruit motor units is obviously inuenced by the subjects cooperation. Poor recruitment could be the result of a subjects unwillingness to cooperate, inability to follow commands, misunderstanding of the required task, or pain. However, despite these difculties, the needle examination is still potentially useful as the motor unit recruitment frequency and recruitment ratio are not inuenced by the previously mentioned volitional factors.

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. A major mismatch, such as when a macro-EMG needle with a large pickup area is used to record from a small intrinsic or foot muscle, could result in a very noisy baseline from the ring of neighboring muscles. The site of needle insertion in relation to the motor endplate and the angle of insertion in relation to the muscle ber plane can also affect the conguration and duration of the motor unit action potential. Finally, the amplitude of a motor unit action potential recorded by concentric or monopolar needle electrode is highly inuenced by the muscle bers in the immediate vicinity of the recording surface. This is particularly likely to occur in conditions in which the ber density is increased, as is often the case in many muscle diseases. Therefore, the amplitude measured by a macro-EMG needle electrode more accurately reects the overall size of the motor unit. Finally, compared with bipolar electrodes, duration of the motor unit action potential is longer when recorded with concentric and monopolar electrodes.

Fatigue and Other Physiological Factors


Muscle fatigue may occur after prolonged contractions, sometimes required when one attempts to record a large number of motor unit action potentials for quantitative analysis. Fatigue can induce changes in the conguration of the motor unit action potentials and alter their recruitment pattern and ring rate (Maton, 1981; Bigland-Ritchie et al., 1986; Dorfman et al., 1990; Miller et al., 1996; Christova and Kossev, 1998; Grifn et al., 1998). Hyperventilation and limb ischemia could lead to fasciculation potentials in otherwise healthy individuals.

References
Adrian ED, Bronk DW: The discharge of impulses in motor nerve bres. Part II. The frequency of discharge in reex and voluntary contractions. J Physiol 1929;67:119151. Albers JW, Allen AA 2d, Bastron JA, Daube JR: Limb myokymia. Muscle Nerve 1981;4:494504. Barkhaus PE, Nandedkar SD: Recording characteristics of the surface EMG electrodes. Muscle Nerve 1994;17:13171323. Bigland-Ritchie B, Cafarelli E, Vollestad NK: Fatigue of submaximal static contractions. Acta Physiol Scand Suppl 1986;556:137 148. Brown WF: The Physiological and Technical Basis of Electromyography. Boston, Butterworth, 1984.

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Peripheral Motor Sensation


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