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DISORDERS OF NEUROMUSCULAR TRANSMISSION
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relatively low amplitude, during attempts at maximal voluntary contraction The latter may mimic a nascent pattern seen in severe denervation and early reinnervation Careful observation may demonstrate a slight increase in the overall amplitude of the electrical activity with prolonged muscle activation, but this is a dif cult impression to quantitate As with repetitive stimulation, the same panoramic selection of muscles should be tested including proximal and distal muscles in both upper and lower extremities as well as those innervated by cranial nerves As the bulbar muscles (eg, masseter, orbicularis oculi and nasalis, hypoglossus, and trapezius muscles) are usually affected rst, these tend to demonstrate greater degrees of abnormality than their limb counterparts Pathological MUAP changes in these muscles may be dif cult to detect, however, as MUAPs in facial muscles are small under normal circumstances as a result of their low innervation ratio With symptom progression and increasing time from disease onset (greater than 10 days), there may be a signi cant reduction in both the numbers and the duration/amplitude of MUAPs and an increased prevalence of positive sharp waves and brillation potentials This is especially true for profound disease in the proximal limb and cranial muscles After several months, there tends to be a rather complete electrophysiologic recovery with respect to MUAP parameters The morphological changes of MUAPs in botulism are explained by mechanisms similar to many myopathies or other DNMT As NMT fails in muscle bers that are in close proximity to the needle electrode, their contribution to the amplitude of the MUAP diminishes As NMT fails in myo bers belonging to that same motor unit but at the periphery of the recording radius of the needle electrode, their contributions to the duration of the MUAP are lost As the synchrony between all of the single muscle ber action potentials contributing to that MUAP is altered by NMT failure in random bers, MUAP polyphasia may occur As NMT failure in a myo ber may effectively denervate it, abnormal spontaneous activity in the form of brillation potentials and positive waves may occur The small number of patients studied with SFEMG allow only a cursory description of what is typical for botulism363,369,378,387,393 395 Predictably, abnormal (increased) jitter values and blocking can be observed early in the disease in 40 50% of SFEMG studies It would appear that, unlike MG, the degree of blocking is somewhat independent of the severity of jitter The mechanism for this is uncertain, although it has been hypothesized that it may be related in part to the acuity of disease onset and the associated impaired quantal content A nding in botulism that is similar to LEMS is the tendency for jitter values to decline in association with an increased frequency of muscle ber action potentials This in all probability due to the same physiological fac-
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tors that lead to electrophysiologic increment and postexercise facilitation described above Fiber density may be relatively low on the initial SFEMG study and may increase as the patient s condition improves396 This is likely a result of the reduced probability of ber pair detection due to failure of NMT in one or both members of a pair that may otherwise be captured under normal conditions As a result, voluntary SFEMG may become more time consuming, and stimulated SFEMG may represent a more ef cient means to assess jitter and potential blocking There have been a few reported cases of increased ber density coincident with patient recovery, which appear to persist, at least for the relatively limited reported period of follow-up262,286,397 Both the severity of the disease and the method of NMJ recovery in botulism may explain these two disparate ndings Reduced ber density may be seen in relatively mild cases of disease where a limited number of NMJs are affected The abnormality is suf cient to reduce the probability of nding two single ber action potentials belonging to the same motor unit within the recording radius of the needle Disease severity may be insuf cient to provoke signi cant motor unit remodeling or what is effectively a denervating and reinnervating disorder that may occur in more severe cases In this latter situation, recovery of the more severely affected NMJs leads to a more signi cant loss of multiple NMJs Substantial nerve terminal sprouting may be required to restore NMJ transmission This provides a potential explanation for the histological changes of denervation atrophy previously described, the increased ber density measurements that represent one of their EDX correlates, and the reduced MUAP recruitment with vigorous attempts to activate a muscle The value of SFEMG is similar to that of other DNMT, ie, an increased sensitivity in the detection of minor changes in NMT before CMAP amplitude reduction or responses to repetitive stimulation become evident In patients with suspected botulism, SFEMG is usually reserved for individuals in whom routine conduction studies and repetitive stimulation and postexercise techniques are nondiagnostic Again, it should be emphasized that the enhanced sensitivity of SFEMG comes with the price of diminished speci city SFEMG abnormalities are therefore at best supportive and not diagnostic of botulism
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