Format

Send to

Choose Destination
Neurology. 2016 May 31;86(22):2085-93. doi: 10.1212/WNL.0000000000002723. Epub 2016 May 4.

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

Author information

1
From the Department of Neurology (D.L.H.K., T.C., F.J.M.), Massachusetts General Hospital, Boston; Department of Neurology (D.L.H.K.), Academic Medical Center, Amsterdam, the Netherlands; Department of Neurology (S.C.), University of Pennsylvania, Philadelphia; Department of Neurology (S.S.M., G.M.H., B.M.), Washington University School of Medicine, St. Louis, MO; Department of Neurology (A.V., A.K.Y., P.B.), Johns Hopkins University School of Medicine, Baltimore, MD; Department of Neurology (J.P.K.), Brigham and Women's Hospital, Boston; Harvard Medical School (J.P.K., F.J.M.), Boston, MA; and Department of Biostatistics (D.C.B., M.C.), University of Washington, Seattle.
2
From the Department of Neurology (D.L.H.K., T.C., F.J.M.), Massachusetts General Hospital, Boston; Department of Neurology (D.L.H.K.), Academic Medical Center, Amsterdam, the Netherlands; Department of Neurology (S.C.), University of Pennsylvania, Philadelphia; Department of Neurology (S.S.M., G.M.H., B.M.), Washington University School of Medicine, St. Louis, MO; Department of Neurology (A.V., A.K.Y., P.B.), Johns Hopkins University School of Medicine, Baltimore, MD; Department of Neurology (J.P.K.), Brigham and Women's Hospital, Boston; Harvard Medical School (J.P.K., F.J.M.), Boston, MA; and Department of Biostatistics (D.C.B., M.C.), University of Washington, Seattle. fmateen@partners.org.

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.

PMID:
27164698
DOI:
10.1212/WNL.0000000000002723
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for HighWire
Loading ...
Support Center