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mental response that is identical to that described in MG338,341,345,346,350 It is sometimes dif cult to appreciate the decrement if the baseline amplitude is too low to begin with Decrement on 3 Hz stimulation has been demonstrated in the abductor digiti quinti (ADQ) in 98%, APB in 98%, EDB in 84%, and trapezius in 89% of cases346 Following a brief maximal exercise period of 10 seconds, there is a blunting of the decrement in response to 2 3 Hz stimulation, resulting in increased amplitudes of the initial as well as subsequent responses This postexercise facilitation effect lasts approximately 20 30 seconds If the muscle is exercised for 1 minute, ie, postexercise exhaustion, 2 3 Hz repetitive stimulation produces a decrement that peaks at 3 5 minutes and exceeds that observed in the resting state Rarely, postactivation exhaustion may last for up to 20 minutes351 There is no correlation between patients with respect to severity of disease and magnitude of decrement, increment, or amplitude of the CMAP at rest In other words, if patients have a CMAP amplitude of 300 V at rest that increments to 8 mV, they do not have more or less severe disease than a patient who has a resting CMAP of 1 mV that increments to 6 mV Insertional activity in LEMS is normal or slightly reduced Positive sharp waves and brillation potentials are usually absent in patients with LEMS Minor membrane instability may be observed131,192,194,211,258 260,332,333,334,337,343 Abnormalities of MUAP morphology are apparent in weak muscles if carefully assessed An effective loss of muscle bers per motor unit due to neuromuscular blockade results in shorter-duration and lower-amplitude MUAPs As a consequence of this effective reduction in MUAP size, the twitch tension of individual motor units decline and compensatory early recruitment may occur Additionally, fewer active muscle bers produce less overlap or synchrony of component single ber action potentials, increasing the percentage and severity of polyphasic MUAPs Motor unit instability is readily evident if sought for With continued contraction, the amplitude of the interference pattern increases and the amount of MUAP variability declines as NMT improves in response to the same postexercise facilitation phenomenon described above Predictably, both volitional and stimulated SFEMG evaluations of patients with LEMS yield abnormal results45,196,225,226,230 232,258,259,260,295 297,334,335,338 340,340, 344,347 Jitter values in patients with LEMS are signi cantly elevated and statistically exceed that observed in MG In essentially all NMJs examined, irrespective of muscle chosen, markedly abnormal jitter values are evident This is disparate from MG where a spectrum of jitter values from normal to highly abnormal exists within and between individual muscles Unlike MG, the jitter in patients with LEMS is not dependent on the degree of weakness in a particular muscle Blocking is often more prevalent and severe in LEMS in comparison to.

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