Acute Disseminated Encephalomyelitis followed by Optic Neuritis: A Rare Syndrome of Uncertain Treatment and Prognosis

Abstract

Aim Acute Disseminated Encephalomyelitis followed by optic neuritis (ADEM-ON), first described in 2013, is a rare demyelinating syndrome, typical of the pediatric age. We conducted a mini review of the existing literature, focusing on clinical, laboratory, radiological, therapeutic, and prognostic aspects in order to improve the identification of new cases.

Methods We searched PubMed and Cochrane Library for studies on ADEM-ON between 2013 and 2018.

Results Examination of the reported cases (three case reports and eight observational studies) established the following features. Time between ADEM and ON is highly variable. Almost all patients show antimyelin oligodendrocyte glycoprotein antibody (MOG-abs) seropositivity. High-dose intravenous steroid and plasmapheresis efficacy is reported for the acute phase; oral prednisone and other maintenance drugs may be useful in avoiding relapses. The clinical history may lead to a complete recovery but also to residual deficits.

Conclusion MOG-abs detection strongly supports ADEM-ON diagnosis, confirming this entity as part of MOG-abs spectrum disorder. Owing to the very small number of cases so far reported, predicting clinical evolution is very difficult.

Keywords

acute disseminated encephalomyelitis - optic neuritis - antimyelin oligodendrocyte glycoprotein antibodies - pediatric demyelinating syndromes

Author Contributions

M.S. and A.N.P. equally contributed to this study; they conceived and collected the data and M.S. wrote the manuscript. M.F. contributed in the literature review. R.P., A.N.T.P., and M.M. helped in drafting the manuscript. L.M. supervised the research. All the authors reviewed and edited the manuscript.

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