Acute disseminated encephalomyelitis in 228 patients: A retrospective, multicenter US study. (31st May 2016)

• Record Type: Journal Article
• Authors: Koelman, Diederik L.H.; Chahin, Salim; Mar, Soe S.; Venkatesan, Arun; Hoganson, George M.; Yeshokumar, Anusha K.; Barreras, Paula; Majmudar, Bittu; Klein, Joshua P.; Chitnis, Tanuja; Benkeser, David C.; Carone, Marco; Mateen, Farrah J.
• Is Part Of: Neurology. Volume 86:Number 22(2016)
• Publication Date: 2016-05-31
• Subjects: Neurology -- Periodicals; Neurology -- Periodicals; Neurologie -- Périodiques; 616.8
• DOI: 10.1212/WNL.0000000000002723
• Languages: English
• ISSNs: 0028-3878

Abstract

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 = 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 = 0.007), in whom MS was diagnosed more often. Encephalopathy at initial presentation (hazard ratio [HR] 0.383, p = 0.001), male sex (HR 0.394, p = 0.002), and increasing age at onset (HR 0.984, p = 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.