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TESTING IN NEUROMUSCULAR DISEASE
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primarily measures of conduction speed and therefore of myelin function Although both conduction velocity and distal latency measure conduction speed, these are reported separately in motor conduction studies for a number of reasons The distal latency re ects conduction along different segments of nerve (wrist to hand or ankle to foot) than the conduction velocity (elbow to wrist or knee to ankle) In certain pathological conditions, one parameter may be abnormal whereas the other remains unaffected Distal latency and conduction velocity are reported differently for purposes of technical accuracy Distal latency measures not only nerve conduction but neuromuscular transmission time as well Additionally, terminal nerve twigs attenuate in diameter and have a conduction velocity that does not accurately re ect conduction speed in the more proximal nerve The last parameter to be assessed on a more subjective basis is CMAP morphology Although morphological changes can be measured by comparing ratios of CMAP duration to amplitude, these are usually observational and descriptive in nature Subtle changes may occur in normal individuals when CMAPs are obtained from stimulation at different points along the course of a nerve These changes consist of amplitude reduction and increased waveform duration, with relative preservation of area under the curve With this physiological dispersion, the CMAP amplitude with proximal stimulation should never drop below 80% of that obtained from the most distal stimulus site Nerve root stimulation sites provide the notable exception to this rule5 More dramatic reductions of CMAP amplitude, particularly over short segments of nerve, should be interpreted as either demyelinating pathology or technical error (Fig 2 2a and b) CMAP afterdischarges represent one other potential alteration of CMAP morphology When present, these repetitive CMAPs follow a single supramaximal nerve stimulus These appear as multiple negative peaks in the immediate aftermath of the initial CMAP detectable either with routine motor conductions or with F wave assessment Afterdischarges are not uniform with consecutive stimuli and have much smaller amplitudes than the initial supramaximal response (Fig 2 3) These occur rarely, typically in disorders in which nerve or muscle depolarization persists or repolarization is delayed Their value is their speci city and the limited number of disorders with which they are known to associate with This differential diagnosis includes disorders of nerve potassium channels such as Issacs syndrome,6 muscle channelopathies (eg, paramyotonia congenita (PMC), myotonia congenita, and potassium-aggravated myotonia), or disorders in which there is prolonged cholinergic activity at neuromuscular junctions7,8 Toxic exposures to organophosphates or congenital acetylcholinesterase de ciency are the most notable examples of the latter9
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Figure 2 2 (A) Short segmental incremental stimulation in normal individual demonstrating identical CMAP (compound muscle action potential) waveforms with equivalent spacing between consecutive waveforms and (B) ulnar neuropathy at the elbow with focal demyelination with conduction block (amplitude reduction between responses 3 and 4), focal slowing (widening baseline interval between responses 3 and 4), and mild temporal dispersion (increased CMAP duration between responses 3 and 4)
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SECTION I
APPROACH TO PATIENTS WITH NEUROMUSCULAR DISEASE
Sensory Nerve Conductions
Sensory conduction studies may be performed percutaneously They are more commonly performed by attaching the same or similar recording electrodes on the surface of the skin over a sensory or mixed nerve The tested nerve is then stimulated at either a more proximal or a distal location than the recording site The former technique is described as antidromic, as the stimulus travels in the direction opposite to that of normal centripetal physiologic conduction in sensory nerve bers With stimuli delivered distal to the recording site, conduction is considered orthodromic Nerves routinely studied include the median, ulnar, dorsal cutaneous ulnar, radial, medial antebrachial cutaneous, lateral antebrachial cutaneous, sural, and super cial peroneal The lateral femoral cutaneous, saphenous, and medial and lateral plantar nerves are less frequently tested These latter studies are fairly easy to obtain in the young, healthy, slender, and nonedematous but can be technically dif-
cult in those with the opposite characteristics Unlike motor conductions, SNAPs are measured in microvolts rather than millivolts, making them technically more dif cult for an inexperienced technician or physician to obtain In many laboratories, only two parameters are measured: SNAP amplitude and distal latency or conduction velocity (Fig 2 4) As there are no neuromuscular junctions to contend with in sensory nerves, both the distal latency and the conduction velocity are measures of nerve conduction speed, differing only in the segment of nerve tested Either or both parameters may be reported With motor conductions, there are some disorders in which distal latencies are prolonged disproportionate to forearm or leg conduction velocities As there are few, if any, recognized conditions in which conduction speed is consistently more affected in one segment of sensory nerves than another, it can be argued that the reporting of distal latency as the sole measurement of sensory conduction speed is adequate With motor
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