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of testing have their main use in the study of amyotrophic lateral sclerosis (ALS) and related disorders As described in Chap 2, by applying repetitive electrical stimuli to a peripheral nerve, the sensory evoked responses can be recorded from sites along the nerve and plexus as well as in central pathways (the thalamus and somatosensory cortex) These evoked potential tests nd their main use in the diagnosis of multiple sclerosis and in disorders of the sensory nerve roots as discussed in Chaps 2 and 36 Other special nerve conduction techniques are available in many laboratories For details of their performance and interpretation the reader is referred to specialized texts on the subject Discussion of magnetic stimulation, collision techniques, quantitative EMG, etc, can be found in several monographs, such as the ones by Kimura, by Aminoff, and by Brown and Bolton
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Repetitive Motor Nerve Stimulation (Jolly Test)
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This test of the function of the neuromuscular junction is based on Jolly s observation in 1895 that in myasthenia gravis the strength of muscular contractions progressively declines in response to a train of stimuli By adjusting the amplitude of a stimulus over a nerve to supramaximal range, a maximal CMAP may be obtained for each stimulus With repeated stimuli, each response will have the same waveform and amplitude until fatigue supervenes In a healthy individual, the response follows each stimulus even with rates of stimulation up to 25 per second for periods of 60 s or more before a decrement of the CMAP appears In certain disorders, notably myasthenia gravis, a train of 4 to 10 stimuli at rates of 2 to 5 per second (optimally 2 to 3 per second), the amplitude of the motor potentials decreases and then, after four or ve further stimuli, may increase slightly (Fig 45-4A) A progressive reduction in amplitude is most likely to be found in proximal muscles, but these are not easily stimulated for which reason the locations most commonly used for clinical testing are the accessory nerve in the posterior triangle of the neck (trapezius), the ulnar nerve (hypothenar muscle), the median nerve at the wrist (thenar muscle), and the facial nerve and orbicularis oculi muscle A decrement of 10 percent or more denotes a failure of a proportion of the neuromuscular
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junctions that are being stimulated The sensitivity of the procedure is improved by rst exercising the tested muscle for 30 to 60 s The induced failure of neuromuscular transmission in myasthenia is similar to the one produced by curare and other nondepolarizing neuromuscular blocking agents, and both cases can be partially corrected with anticholinesterase drugs such as neostigmine and edrophonium Similar but lesser decremental responses may occur in poliomyelitis, ALS, and certain other diseases of the motor unit or motor nerve, particularly those resulting in the growth of reinnervating nerve twigs The myasthenic syndrome of Lambert-Eaton, often associated with oat-cell carcinoma of the lung, is characterized by a presynaptic blockage of acetylcholine release and produces the opposite defect of neuromuscular transmission to the one recorded in myasthenia gravis During tetanic stimulation (20- to 50-per-second repetitive stimulation of nerve), the muscle action potentials, which are small or practically absent with the rst stimulus, increase in voltage with each successive response until a more nearly normal amplitude is attained (see Fig 45-4B) Exercising the muscle for 10 s before stimulation will cause a similar posttetanic facilitation (200-fold increases are not uncommon) A less important decremental response to slow stimulation may occur, but it is dif cult to discern because of the greatly diminished amplitude of the initial responses Neostigmine has little effect on this phenomenon, but it may be reversed by guanidine and 3,4-diaminopyridine, which stimulate the presynaptic release of ACh The effects of botulinum toxin and of aminoglycoside antibiotics are similar, ie, being active at the presynaptic membrane, they produce an incremental response at high rates of stimulation The single- ber EMG, discussed in a later section, is an even more sensitive method of detecting failure of the neuromuscular junction
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Needle Examination of Muscle (Electromyography)
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This technique requires the use of monopolar or concentric bipolar needle electrodes, which are inserted into the body of the muscle to record the electrical activity generated by contraction With concentric needle electrodes, the tip of the wire that runs in the hollow of the needle is in proximity to many muscle bers belonging to several different overlapping motor units; this is the active recording electrode The shaft of the needle, in contact over most of its length with intercellular uid and many other muscle bers, serves as the reference electrode With monopolar electrodes, the uninsulated needle tip is the active electrode, while the reference electrode may be another monopolar needle electrode placed in subcutaneous tissue or a surface electrode on the skin overlying the muscle Patients almost invariably nd this portion of the test uncomfortable and should be prepared by a description of the procedure Rapid and brief needle insertion by the skilled examiner makes the test more tolerable As the electrical impulse travels along the surface of the muscle toward the recording electrode, a positive potential is recorded on the oscilloscope, ie, the recorded signal is de ected downward by convention (at A in Fig 45-5) When the depolarized zone moves under the recording electrode, it becomes relatively negative and the beam is de ected upward (at B) As the depolarized zone continues to move along the sarcolemma, away from the recording electrode, the current begins to ow outward through the membrane toward the distant depolarized region, and the recording electrode
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Figure 45-4 Compound action potentials evoked in hypothenar muscles by electrical stimulation of the ulnar nerve at the wrist A Patient with myasthenia gravis typical pattern of decrement in rst four responses followed by slight increment At this rate of stimulation (4 per second), the decrement in response does not continue to zero B Patient with LambertEaton syndrome and oat-cell carcinoma typical marked increase toward normal amplitude with rapid repetitive stimulation (20 per second) Horizontal calibration: 250 ms
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