Treatment

The first-line acute treatment for ADEM is immunosuppression with high-dose IV glucocorticoids. The standard protocol is methylprednisolone at 30 mg/kg/day IV (maximum 1000 mg/day) for 3 to 5 days, followed by an oral prednisone taper for 4 to 6 weeks. Early discontinuation of steroids may increase the risk of relapse. (1)

These can be started simultaneously with acyclovir or antibiotics on the initial patient presentation. If a patient is not improving or is responding poorly to glucocorticoid treatment, IV immunoglobulin is a good second-line therapy. The dose is 2 g/kg for 2 to 5 days. Plasma exchange with 3 to 7 exchanges, or cyclophosphamide, is used in refractory cases. (2)

The disease is associated with a 20% mortality. Cognitive impairment is reported in up to 56% of children after an ADEM. (3)

Reference

1. Cole J, Evans E, Mwangi M, Mar S. Acute Disseminated Encephalomyelitis in Children: An Updated Review Based on Current Diagnostic Criteria. Pediatr Neurol. 2019 Nov;100:26-34.
2. Yae Y, Kawano G, Yokochi T, Imagi T, Akita Y, Ohbu K, Matsuishi T. Fulminant acute disseminated encephalomyelitis in children. Brain Dev. 2019 Apr;41(4):373-377.
3. Deiva K et al. Risk factors for academic difficulties in children with myelin oligodendrocyte glycoprotein antibody-associated acute demyelinating syndromes. Dev Med Child Neurol. 2020 Sep;62(9):1075-1081.