Acute disseminated encephalomyelitis in 228 patients: A retrospective, multicenter US study

Diederik L.H. Koelman, Salim Chahin, Soe S. Mar, Arun Venkatesan, George M. Hoganson, Anusha K. Yeshokumar, Paula Barreras, Bittu Majmudar, Joshua P. Klein, Tanuja Chitnis, David C. Benkeser, Marco Carone, Farrah J. Mateen

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36 Scopus citations

Abstract

Objective: To analyze the range of demographic, clinical, MRI, and CSF features of acute disseminated encephalomyelitis (ADEM), a rare, typically monophasic demyelinating disorder, and analyze long-term outcomes including time and risk factors for subsequent clinical events as well as competing diagnoses. Methods: We performed a retrospective, multicenter study in 4 US academic medical centers of all patients clinically diagnosed with ADEM. Initial presentation of pediatric and adult ADEM and monophasic and multiphasic disease were compared. The Aalen-Johansen estimator was used to produce estimates of the probability of transitioning to a multiphasic diagnosis as a function of time since initial diagnosis, treating death and alternative diagnoses as competing risks. Results: Of 228 patients (122 children, age range 1-72 years, 106 male, median follow-up 24 months [25th-75th percentile 6-67], 7 deaths), approximately one quarter (n 5 55, 24%) experienced at least one relapse. Relapsing disease in children was more often diagnosed as multiphasic ADEM than in adults (58% vs 21%, p 5 0.007), in whom MS was diagnosed more often. Encephalopathy at initial presentation (hazard ratio [HR] 0.383, p 5 0.001), male sex (HR 0.394, p 5 0.002), and increasing age at onset (HR 0.984, p 5 0.035) were independently associated with a longer time to a demyelinating disease relapse in a multivariable model. In 17 patients, diagnoses other than demyelinating disease were concluded in long-term follow-up. Conclusions: Relapsing disease after ADEM is fairly common and associated with a few potentially predictive features at initial presentation. Age-specific guidelines for ADEM diagnosis and treatment may be valuable, and vigilance for other, mostly rare, diseases is imperative.

Original languageEnglish
Pages (from-to)2085-2093
Number of pages9
JournalNeurology
Volume86
Issue number22
DOIs
StatePublished - May 31 2016

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    Koelman, D. L. H., Chahin, S., Mar, S. S., Venkatesan, A., Hoganson, G. M., Yeshokumar, A. K., Barreras, P., Majmudar, B., Klein, J. P., Chitnis, T., Benkeser, D. C., Carone, M., & Mateen, F. J. (2016). Acute disseminated encephalomyelitis in 228 patients: A retrospective, multicenter US study. Neurology, 86(22), 2085-2093. https://doi.org/10.1212/WNL.0000000000002723