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transiently augmented by increased quantal release from the presynaptic terminal As will be described in more detail below, this is a consequence of the increased calcium concentration within the presynaptic terminal that has not had a chance to dissipate between consecutive stimuli because of the short stimulus interval between stimuli The facilitation of quantal release provoked by either exercise or fast repetitive stimulation is limited in duration and quickly replaced by exhaustion if either the stimulus train or the exercise exceeds 30 60 seconds It should be emphasized that patients with postsynaptic DNMT such as autoimmune MG have CMAP amplitudes that are typically normal at rest, which cannot be augmented by either of the facilitation techniques described above The only role of postexercise facilitation in MG is to repair a decrement that has been previously created by slow repetitive stimulation Although more cumbersome, a decrement may be repaired pharmacologically as well with the use of the cholinesterase inhibitor, edrophonium Although not usually required for diagnostic purposes, the decrement can be readily recreated following repair by applying an additional 1 minute of isometric exercise followed by an additional train of slow repetitive stimulation The muscles chosen for this exercise represent a compromise between muscles that are technically easiest to study (better-tolerated, less movement artifact) and those that have the highest diagnostic yield Intrinsic hand muscles may fail to demonstrate a signi cant decremental response in 50 68% patients with moderately severe MG272,276 Proximal muscles, eg, the biceps, deltoid, and the trapezius, have higher yields Erb s point stimulation with deltoid recording may be 80 90% sensitive in patients with myasthenia, but brachial plexus stimulation is both uncomfortable and technically dif cult in view of the movement it creates Accessory nerve stimulation has similar sensitivity and is usually better tolerated with an easier to establish stable baseline278 Movement artifacts may be averted by strategies that diminish actual body movement An arm board can be used to immobilize the elbow with musculocutaneous nerve stimulation in the axilla Patients can sit on their hands during axillary nerve stimulation A belt or cloth loop connecting ipsilateral hand with the foot of the extended leg may provide additional stabilization for trapezius recordings With stimulation of the common peroneal nerve while recording from the tibialis anterior, stabilizing the ankle with an ankle foot orthosis may improve the quality of the result Facial nerve activation with recordings from the orbicularis oculi or nasalis muscles has a higher yield than limb muscles Movement can be a signi cant problem in some patients In view of the proximity of the stimulating electrode to the contracting muscles, there is a signi cant potential for movement of the stimulating electrodes Using electrodes securely taped to the angle of the jaw over the facial nerve with a.

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plastic pen or pencil applying pressure to the middle of the bar electrode is one way to minimize this problem Historically, regional applications of both ischemia and d-tubocurarine curare were used in addition to slow repetitive stimulation, in an effort to increase diagnostic yield in MG As these are cumbersome and potentially risky maneuvers, SFEMG represents the preferred EDX alternative in most EMG laboratories where slow repetitive stimulation is nondiagnostic Slow repetitive stimulation is estimated to be abnormal in 37 62% of individuals with MG43,258 260,262,274,279 286 Stimulation of a proximal muscle increases the percentage of abnormal responses to 62 77% In patients with ocular MG, repetitive stimulation of a distal upper extremity nerve yields positive results in only up to 35% of patients Adding a proximal nerve increases the yield slightly to 45% Patients who are MuSK antibody positive tend to have both a lesser incidence and a lesser degree of decrement in limb muscles compared to other MG populations73,85 A higher yield of abnormal decrement will be found in facial muscles in the anti-MuSK patient population82 Estimates of incidence of abnormal decrement in these patients range from one-third to one-half in limb muscles as opposed to 50 85% in facial muscles88 All of the aforementioned information pertaining to the EDX assessment of NMT applies to children and adults alike In normal infants, the limited data available suggest that with stimulation rates between 1 and 2 Hz, there is no alteration in the CMAP287 289 Repetitive stimulation at rates between 2 and 5 Hz yields variable results, with some normal infants demonstrating a decrement and others revealing no change At 5 Hz, most term infants should not demonstrate an alteration in the sequentially recorded CMAPs, although a few may demonstrate a mild (11 17%) increment with an occasional decrement being noted If a 10-Hz train of stimuli is applied for 15 seconds, about half of children examined who are 39 weeks or older may reveal an increment approaching 14% When a 15-second 20-Hz stimulation train is applied, most children reveal a decrement of about 24%, while premature infants (<38 weeks) have a larger decrement (about 32%) A few term infants may demonstrate an initial increment for several responses followed by the anticipated decrement If repetitive stimulation is delivered at 50 Hz for 15 seconds, all term as well as premature infants demonstrate a decrementing CMAP response of approximately 50% with premature infants having larger decrements of about 77% These ndings suggest that term infants and especially premature infants have a reduced NMJ reserve capacity, especially at the higher rates of stimulation It is certainly possible to examine infants with repetitive stimulation techniques as long as the above ndings are kept in mind This is particularly relevant following 50 Hz.

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