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Site no 1: 2: 3: 4: Lat ms 28 25 67 00 Amp mV 23 75 28 * 00 Area ms*mV 67 218 90 00 Stimulus mA 2000 2000 2500 000

Figure 2 8 Incremental response to brief (10 seconds) exercise in patient with LEMS (A) (trace 1 ulnar CMAP at baseline stimulating at wrist, trace 2 ulnar CMAP immediately after 10 seconds of isometrically resisted nger abduction stimulating at wrist, trace 3 ulnar CMAP 1 minute later stimulating at elbow) Incremental response to 20 Hz fast repetitive stimulation (B) CMAP, compound muscle action potential; LEMS, Lambert Eaton myasthenic syndrome

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following 10 15 seconds of exercise to look for postexercise facilitation or decrement repair If a decrement is not demonstrable at baseline, repeating the train once a minute for 5 minutes following 1 minute of exercise applied to that muscle may improve diagnostic yield This phenomenon is referred to as postexercise exhaustion Presynaptic DNMTs also have a decrement with low rates of repetitive stimulation but are distinguished from MG on the basis of low baseline CMAP amplitudes Although it does not de ne a presynaptic DNMT, a decremental response to slow repetitive stimulation may be easier to demonstrate early in the course of these disorders than its incremental counterpart, as the baseline CMAP amplitude may not have declined to the point where a signi cant increment is possible

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DNA mutational analyses for the nondystrophic ion channel disorders are not readily available Short- and long-exercise tests have been developed in an attempt to identify the existence of and to differentiate between these sodium, chloride, and calcium channel disorders7,8 The short- and long-exercise tests are variations of standard motor NCS, typically using the ulnar nerve or common peroneal nerve recording from the abductor digiti minimi or extensor digitorum brevis muscles, respectively Both exercise tests require careful attention to uniform patient positioning, limb temperature, muscle relaxation, and stimulus intensity The short-exercise test is performed by having a patient isometrically exercise the abductor digiti minimi or extensor digitorum brevis for 10 seconds, followed by sequential CMAP amplitude measurements obtained by single supramaximal (and nonvarying) stimuli delivered immediately after exercise and at 10-second intervals for a total of 50 seconds Typically, repeated stimuli at 10-second intervals are delivered on a preliminary, pre-exercise basis to insure a stable baseline After a 10second rest between trials, two subsequent trials are recommended, which provide further discriminating value between different mutations and resultant phenotypes as subsequently described This test has been further modi ed by using the same algorithm following limb cooling8 In normal individuals, a transient increase in CMAP amplitude approximating 5% is noted immediately post exercise, without change in waveform morphology The CMAP reverts to its baseline amplitude within 10 seconds post exercise In studied normal patients, variations of the initial postexercise CMAP response were considered normal if within the 10% to +20% range of baseline CMAP amplitude, although no patient studied had a response greater than +14% or less than 6%7 Afterdischarges, as described previously in this chapter and subsequently in this section, are not expected in normal individuals In chloride channel disorders associated with myotonia congenita phenotype (MC), and to a lesser extent in sodium channel disorders producing PC, two distinct abnormalities can be observed7 One is a decline in postexercise CMAP amplitude, which is commonly in the 50% range and persists on average for 20 40 seconds in MC and in excess of 1 minute in PC This phenomenon.

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