Acute Disseminated Encephalomyelitis in Children S. N. Krishna Murthy, MD*‡§; Howard S. Faden, MD‡; Michael E. Cohen, MD*‡§; and Rohit Bakshi, MD*¶# ABSTRACT. Objective. To describe the epidemio- logic, clinical, neuroimaging, and laboratory features; treatment; and outcome in a cohort of children with acute disseminated encephalomyelitis (ADEM). Methods. A 6-year retrospective chart review of chil- dren with the diagnosis of ADEM was conducted. Results. Eighteen cases were identified. Sixteen pa- tients (88%) presented in either winter or spring. Thir- teen children (72%) had a recent upper respiratory tract illness. Patients presented most often with motor deficits (77%) and secondly with altered consciousness (45%). Spinal fluid abnormalities occurred in 70%. Despite rig- orous microbiologic testing, a definite microbiologic di- agnosis was established only in 1 child with Epstein-Barr virus disease and probable or possible diagnoses in 3 children with Bartonella henselae, Mycoplasma pneu- moniae, or rotavirus disease. Brain magnetic resonance imaging identified lesions in the cerebral cortex in 80%, in subcortical white matter in 93%, in periventricular white matter in 60%, in deep gray matter in 47%, and in brainstem in 47% of patients. Eleven patients (61%) were treated with corticosteroids, and 2 were treated with in- travenous immunoglobulins. All patients survived. Three patients (17%) had long-term neurologic sequelae. Conclusions. Epidemiologic evidence from this study suggests an infectious cause for ADEM. The agent is most likely a difficult-to-diagnose winter/spring respira- tory virus. Magnetic resonance imaging was the neuro- imaging study of choice for establishing the diagnosis and for following the course of the disease. Prognosis for survival and outcome was excellent. Recurrent episodes of ADEM must be differentiated from multiple sclerosis. Pediatrics 2002;110:e0–e0. URL: www.pediatrics.org/cgi/ doi/10.1542/peds.; acute disseminated encephalomyelitis, ADEM, encephalitis, postinfectious encephalitis, encepha- lomyelitis. ABBREVIATIONS. ADEM, acute disseminated encephalomyelitis; CNS, central nervous system; MRI, magnetic resonance imaging; FLAIR, fluid-attenuated inversion recovery; PCR, polymerase chain reaction; EBV, Epstein-Barr virus; Ig, immunoglobulin; TR, repetition time; TE, echo time; NSA, number of signal averages; FOV, field of view; CT, computerized tomography; WBC, white blood cell; CSF, cerebrospinal fluid; IVIG, intravenous gamma- globulin; MS, multiple sclerosis. A cute disseminated encephalomyelitis (ADEM) is considered a monophasic acute demyelinating disorder of the central ner- vous system (CNS) characterized by diffuse neuro- logic signs and symptoms coupled with evidence of multifocal lesions of demyelination on neuroimag- ing. The epidemiology of ADEM has changed since its original description by Lucas 1 in the early 18th century. At that time, ADEM commonly followed common childhood infections such as measles, smallpox, and chickenpox and was associated with significant mortality and morbidity. In a series of case reports in 1931 in The Lancet, McAlpine 2 de- scribed 3 sets of patients with ADEM: 1) postvacci- nation, 2) after infectious fevers such as in measles, and 3) spontaneous. Those with spontaneous and postvaccination ADEM did well despite the lack of antibiotics, steroids, and intensive care facilities, whereas those with an infectious cause fared poorly. A number of recent reports of ADEM in children have confirmed the observations of McAlpine. 3,4 Several articles suggested that improved outcome of ADEM was attributable mainly to the use of steroids; however, evidence for this was mainly anecdotal. 5,6 The purpose of the present study was to review ADEM from a single institution with an emphasis on the relationship of clinical features, microbiology, neuroimaging, and treatment to clinical outcome. Eighteen patients with ADEM were identified. Re- spiratory infections preceded the neurologic presen- tation in the vast majority. Although in most cases a specific cause could not be identified, the outcome was good regardless of treatment. METHODS The inpatient database of Children’s Hospital of Buffalo was broadly searched for patients with the diagnosis of ADEM, viral encephalitis, postinfectious encephalitis, encephalomyelitis, and transverse myelitis. Sixty-seven cases that occurred between Jan- uary 1995 and March 2001 were identified. The diagnosis of ADEM was based on the acute onset of neurologic signs and symptoms together with magnetic resonance imaging (MRI) evi- dence of multifocal, hyperintense lesions on fluid-attenuated in- version recovery (FLAIR) and T2-weighted images. Of the 67 patients, 18 patients fulfilled the diagnostic criteria for ADEM. Clinical information was obtained from the inpatient case records. Microbiologic data were extracted from laboratory re- ports and progress notes in the individual charts. Records main- tained in the microbiology laboratories were reviewed for any tests performed beginning 1 month before admission to the hos- pital and ending 1 month after discharge. The specific tests re- viewed included cultures for bacteria, viruses, and fungi; fluores- cent antibody tests for respiratory viruses; polymerase chain reaction (PCR) tests for enteroviruses, herpes simplex virus, Ep- stein-Barr virus (EBV), and Mycoplasma pneumoniae; enzyme- linked immunosorbent assay for rotavirus; and immunoglobulin From the Departments of *Neurology and ‡Pediatrics, State University of New York at Buffalo, School of Medicine and Biomedical Sciences, Buffalo, New York; Divisions of §Child Neurology and Infectious Diseases, Chil- dren’s Hospital of Buffalo, Buffalo, New York; and ¶Imaging Services and the #Buffalo Neuroimaging Analysis Center, Jacobs Neurological Institute of Kaleida Health, Buffalo, New York. Received for publication Dec 18, 2001; accepted Apr 8, 2002. Reprint requests to (H.S.F.) Division of Infectious Diseases, Children’s Hospital of Buffalo, 219 Bryant St, Buffalo, NY 14222. E-mail: hfaden@upa.chob.edu PEDIATRICS (ISSN 0031 4005). 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