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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.

Isolated cases of foodborne, wound, or inadvertent botulism may escape diagnosis This is, in part, due to its relative rarity and the lack of a single, readily available test that has adequate sensitivity and speci city The diagnosis should be considered in any adult with an acute onset of symptoms suggesting cholinergic dysautonomia, impaired GI motility and weakness, particularly

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If all of the actions in Step 6 were successful, you can probably be comfortable that the system is stable and can report to the IT manager that Jane s machine is back up and running If any of the steps failed, you should use the mouse to select Start | Shut Down In the Shut Down Windows box, select Restart from the drop-down list After the system reboots, you may complete actions 2, 3, and 4 once more Sometimes the forced power down leaves some of the les in a state of ux; restarting shuts the computer down gracefully, properly closing all open les before powering down This should clear everything up and enable the computer to function properly

affecting cranial muscles In infants >3 months of age, hypotonia, impaired sucking, crying, and/or breathing dif culties should raise a similar suspicion398 Edrophonium testing is of limited value A positive response is rarely dramatic and occurs in only a quarter of affected individuals361,399,400 Lumbar puncture may disclose an elevated protein level but its value, like edrophonium testing, is limited by its lack of speci city More speci c evidence of intoxication is sought by analyzing and culturing specimens of serum, gastric contents, stool, wound aspirate, or suspected foods for toxin and organism, respectively Blood should be collected prior to the administration of antitoxin BTX assay is an indirect bioassay that involves injecting mice with one or more of the aforementioned samples If the mouse dies, the injection is repeated with the addition of speci c antitoxins This implicates not only BTX as the cause of death but serves to specify the exact strain The diagnostic yield varies depending on a number of factors including method of inoculation and duration between symptom onset and testing The yield of toxin detection is understandably higher early in the disease, whereas the yield of culture increases with disease duration in untreated patients In general, toxin tests have a yield of approximately 1/3 and are higher in blood and stool than in gastric aspirates Positive cultures may be obtained in a half to two-thirds of cases Unfortunately, these tests are time consuming and costly, typically available only from state laboratories and the CDC Rapid con rmation of toxin detection or culture result does not typically occur398,401,402 Newer, potentially more expeditious and accurate assays are in development Problems associated with their in vivo application have kept them from supplanting the mouse toxin bioassays and anaerobic culture as the diagnostic tests of choice

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impaired NMT as opposed to axonal loss or demyelination as the primary disease mechanism Motor conduction velocities and CMAP amplitudes are typically mildly reduced in paretic upper and lower extremities240,241,403 407 Removal of the tick within several days of clinical presentation results in the prompt resolution of both size and speed of motor and sensory conduction responses Repetitive nerve stimulation at both low and high rates does not reveal either a signi cant decrement or a pathologic increment in the majority of cases, although there have been a few reported cases of an incremental response On closer inspection, these increments probably represent pseudofacilitory responses This phenomenon may also have contributed to the classi cation of tick paralysis as a DNMT in many writings Needle electromyographic evaluation of individuals with acute tick paralysis performed within several days of onset of weakness or after tick removal usually demonstrates only abnormalities of recruitment, reminiscent of any acute neurogenic disorder As such, it supports that tick paralysis, at least in some cases, is more of a disorder of nerve than of NMT Positive sharp waves and brillation potentials are notable for their absence In one apparently outlier case of a child with apparently 50 or more attached ticks, abnormal spontaneous activity in the form of brillation potentials and positive waves appeared within several days of paresis onset405 These abnormal spontaneous potentials persisted for the 6 months this patient was followed

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