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up to 1 to 20 cm in diameter but composed of con uent perivenous lesions The disease could be reproduced in dogs persuasive evidence that one form of acute MS is a variant of ADEM Encephalomyelitis following vaccination against smallpox has been known since 1860, having occurred about once in 4000 vaccinations This disease is now of historical interest only, insofar as smallpox has disappeared as a human illness worldwide In the United States, there have been no new cases for years, and smallpox vaccination is no longer included as part of immunization schedules Recent political events, which have resurrected a national program of vaccination, perhaps may cause the problem to recur if widespread immunizations are undertaken The association of the neurologic disorder with vaccination usually leaves the diagnosis in little doubt, and the characteristic combination of encephalitic and myelitic features will help to distinguish the condition from meningitis, viral encephalitis, and poliomyelitis Rarely, an atypical case may mimic any one of these disorders On occasion, the disease may suggest involvement of nerve roots and peripheral nerves and resemble acute in ammatory polyneuritis (Guillain-Barre syndrome) In fact, the rabies vaccine produced in South America from suckling mouse brain causes this type of peripheral nerve disease more often than encephalomyelitis Mundane inoculations such as those for in uenza or hepatitis must have a very small rate of ADEM, judging from surveillance studies; Ascherio and colleagues were unable to nd any increase in cases among two large studies of nurses who received hepatitis B vaccine The absence of a clear connection of MS to vaccination has already been mentioned The mortality rate of postvaccinal encephalomyelitis is high, between 30 and 50 percent If recovery occurs, it may be surprisingly complete However, a signi cant proportion of patients show residual neurologic signs, mainly in the form of seizures, intellectual impairment, or behavioral abnormalities Treatment With regard to treatment of ADEM, the use of highpotency steroids appears to be the best choice, although controlled trials of this have not been carried out Steroids, given before or immediately upon the appearance of neurologic signs, may modify the severity of experimental allergic encephalomyelitis; this provides the logic for their use in the human counterpart of this disease However, the pathologic process cannot be altered once the rst signs appear Plasma exchange and intravenous immune globulin have also been successful in some fulminant cases (Kanter et al; Stricker et al) but only in a few we have observed.

Acute Necrotizing Hemorrhagic Encephalomyelitis (Acute Hemorrhagic Leukoencephalitis of Weston Hurst)

Startup Modes There are several modes with which you can start a system to allow for troubleshooting The most common modes you will use are discussed briefly in this section:

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This, the most fulminant form of demyelinative disease, affects mainly young adults but also children It is almost invariably preceded by a respiratory infection of variable duration (1 to 14 days), sometimes due to M pneumoniae but more often following a mundane infection or of indeterminate cause The neurologic symptoms appear abruptly, beginning with headache, fever, stiff neck, and confusion These are followed in short order by signs of disease of one or both cerebral hemispheres and brainstem focal seizures, hemiplegia or quadriplegia, pseudobulbar paralysis, and progressively deepening coma Leukocytosis is usually present, sometimes reaching 30,000 cells per cubic millimeter, and the sedimentation rate is elevated The CSF is often under increased pressure; cells vary in number from a few lymphocytes to a polymorphonuclear

ber of other drugs have been utilized with variable success, but these should be considered second-line therapies; these alternatives are discussed by Louis Amantadine also has a modest effect on tremor and may be used as an adjunct The alternate-beat, kinetic-predominant type of essential tremor is more dif cult to suppress but may respond well to clonazepam (Biary and Koller); in our experience, however, this approach has not been as successful Alcohol and primidone have also been less effective in our experience Indeed, the tremor has often been resistant to most attempts at suppression by medication Injections of botulinum toxin can reduce the severity of essential tremor, but the accompanying weakness of arm and hand muscles often proves unacceptable to the patient The same medication injected into the vocal cords can suppress severe voice tremor but caution must be exercised to avoid paralyzing the cords In desperate cases, a thalamic stimulator of the type used to treat Parkinson disease (page 924) has been quite successful and apparently has produced a durable response over many years; details can be found in the small study reported by Sydow and colleagues

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