Multimodal treatment of acute disseminated encephalomyelitis in children

Abstract

The chief treatment options for acute disseminated encephalomyelitis (ADEM) include methylprednisolone (MP), plasma exchange (PE), intravenous immunoglobulin (IVIG). However, there is no evidence-based recommendations for management of ADEM. To identify the prognosis in ADEM after use of different modalities of treatment, a 3-year prospective study of cases presenting with neurological features with a temporal relation to an infection or vaccination or with a presumed etiology as demyelination was undertaken. Investigations to identify the causative agent, magnetic resonance imaging of brain and spinal cord, cerebrospinal fluid studies, electrophysiological studies, blood tests to exclude metabolic and collagen vascular disorders were done. A standard protocol of steroids, failing which (Modified Rankin scale score of four or 5 at end of 3 weeks) IVIG or PE was given. One patient underwent hemicraniectomy. Cases were followed up for 1 year. Telephonic interview was done at 3rd and 5th year. Of the 32 cases (< 18 years), 84% had early complete recovery with MP. One who was on dexamethasone recovered by 1 year. With subsequent PE or IVIG, four cases (13%) had complete recovery. Relapses were restricted to a maximum of three between 6 and 18 months. Prognosis in ADEM can be improved remarkably by early diagnosis and treatment with intravenous MP followed at times by plasmapheresis or IVIG. Hemicraniectomy may be life saving.

Keywords

Plasma exchange - methylprednisolone - immunoglobulin - acute disseminated encephalomyelitis - hemicraniectomy