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DISORDERS OF NEUROMUSCULAR TRANSMISSION
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tumor growth Whether this theoretical risk of immunosuppression in LEMS is a legitimate concern is unknown Unlike MG, there is no role for thymectomy in the treatment of LEMS Plasmapheresis may be helpful in patient with LEMS, but the effect wears off after a few weeks and must be repeated346,776 780 Both clinical and electrical improvement in CMAP amplitudes at rest, following exercise, or in response to high rates of repetitive stimulation may be seen following plasmapheresis777,778 The peak response is observed by about 2 weeks after the treatment, with a diminution in effectiveness by the end of 3 4 weeks As in MG, plasmapheresis is a temporizing measure and patients will need the addition of an immunosuppressive agent if a response is to be sustained IVIG has been noted to be bene cial in small, uncontrolled series of patients with LEMS346,781 Dosing is similar to that outline for MG Prospective, double-blind, controlled trials are necessary to better assess the ef cacy of this treatment
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Intensive care is the foundation of care for the patient with botulism It is undoubtedly the major reason why contemporary mortality rates are less than reported historically Monitoring ventilation and the prompt application of positive pressure ventilation are the most important aspects of surveillance and intervention As with any paralyzing illness, attention to positioning for purposes of comfort, minimizing aspiration risk, improving ventilation, and avoidance of skin break and compression neuropathies are crucial Secretion clearance and the avoidance of malnutrition and deep vein thrombosis are equally important Constipation adds to both the discomfort and the morbidity of the disease Psychological support and a clear understanding of the disease course should be explained to the patients and their families Antitoxin should be considered if administered within 24 hours of symptom onset, while the toxin is still (at least partially) circulating580,585,588 It is not generally recommended for infantile cases Once the toxin enters the nerve terminal, the risk of allergic reaction to this equine-derived biological undoubtedly exceeds any potential bene t The antitoxin, which is available typically from governmental agencies, targets only the exotoxins of the A, B, and E strains of C botulinum A single vial, diluted 10::1 with normal saline, should be administered as a slow IV drip There is no bene t to doubling or repeating the dose Human botulinum immune globulin has a limited role and is approved only for the treatment of infantile botulism It has been shown to reduce the length of hospitalization as well as for the need for mechanical ventilation782
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Guanidine and several aminopyridine derivatives (4-aminopyridine and 3,4 DAP) have the ability to increase the amount of ACh from nerve terminal damaged by BTX in vitro, but human trials have resulted in mixed results as well as disturbing side effects236 Trying to extricate unbound toxin from the GI tract in infantile and foodborne botulism either by lavage or by catharsis is controversial and not routinely used Potential future options for patients affected by botulinum intoxication is the administration of drugs that antagonize the effects of the toxin585,783 With wound botulism, the wound should be debrided Antibiotics generally have no role in most forms of botulism If antibiotics are to be used in patients with wound botulism, they should follow the administration of the antitoxin as organism lysis may lead to signi cant toxin release Consideration should also be given to avoid antibiotics such as the aminoglycosides with neuromuscular blocking properties Recovery is usually satisfactory in all patients, provided they are cared for in a hospital setting from the rst manifestations of the disease In the elderly, associated complications can lead to unavoidable death There are long-term sequelae of fatigue and some mild reduction in respiratory capacity in selected patients Historically, a diagnosis of botulism was associated with an estimated 60% mortality rate Currently, the mortality rate of foodborne and infantile botulism approximates 5%,784,785 whereas that from wound botulism is typically higher and estimated at about 15%402 Patients often complain of marked fatigue, exercise intolerance, general weakness, dry mouth, and shortness of breath, which may persist for over 1 year and seldom allow them to return to full-time work for months607 The return of normal strength seems to precede resolution of these other less objective symptoms
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