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early in the emergency department through pulmonary function testing (in
cooperative children) and close clinical monitoring for fatigue and other clinical
signs of impending failure. Most children will recover with immunomodulatory
therapy (intravenous [IV] immunoglobin, or plasma exchange [PLEX] in more
severe cases), however, recovery times vary and can take months to a year in
some patients. Definitive treatment will require collaboration with critical care
and neurology.
Randomized trials for the treatment of ADEM are lacking, but general
consensus indicates that high-dose IV methylprednisolone (30 mg per kg per day,
maximum 1,000 mg per day) should be initiated early and given for 5 days,
usually followed by an oral taper over 4 to 6 weeks. IV Ig (2 g per kg over 2 to 5
days) is often considered as a second-line agent in cases poorly responsive to
steroids. PLEX is reserved for refractory and particularly severe cases. Prompt
initiation of treatment usually results in excellent outcome, with full recovery in
the majority of cases within days or weeks.
Suggested Readings and Key References
Caffarelli M, Kimia AA, Torres AR. Acute ataxia in children: a review of the
differential diagnosis and evaluation in the emergency department. Pediatr
Neurol 2016;65:14–30.
Thakkar K, Maricich SM, Alper G. Acute ataxia in childhood: 11-year experience
at a major pediatric neurology referral center. J Child Neurol 2016;31(9):1156–
1160.