Acute Disseminated Encephalomyelitis in Children

Abstract

ABSTRACT. Objective. To describe the epidemiologic, 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 children with the diagnosis of ADEM was conducted.Results. Eighteen cases were identified. Sixteen patients (88%) presented in either winter or spring. Thirteen 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 rigorous microbiologic testing, a definite microbiologic diagnosis was established only in 1 child with Epstein-Barr virus disease and probable or possible diagnoses in 3 children with Bartonella henselae, Mycoplasma pneumoniae, 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 intravenous 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 respiratory virus. Magnetic resonance imaging was the neuroimaging 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, encephalomyelitis.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 gammaglobulin; MS, multiple sclerosis.A cute disseminated encephalomyelitis (ADEM) is considered a monophasic acute demyelinating disorder of the central nervous system (CNS) characterized by diffuse neurologic signs and symptoms coupled with evidence of multifocal lesions of demyelination on neuroimaging. 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 described 3 sets of patients with ADEM: 1) postvaccination, 2) after infectious fevers such a...

Prediction of Multiphase Alternative of Acute Disseminated Encephalomyelitis Course Development

“…The recent investigations showed that 4 (13%) of 31 patients with ADEM had manifestations of ADEM relapses, while 10 (40%) of 25 patients had clinically confirmed multiple sclerosis [21]. As it follows from the data of Schwarz, S., et al's [20] [22], developed multiple sclerosis was found in 14 (35%) of 40 patients in 38-month period of observations (which was confirmed using the Poser criteria). In the Alan investigations, 13 (33.3%) of 39 patients faced the replaces of demyelination disease [9] [21].…”

“…The results of observations aimed at the course of this disease for 40 patients with the ADEM diagnosis show that 14 (35%) of them demonstrate developed clinically confirmed multiple sclerosis one year after the primary ADEM episode [20]. Another investigation showed that 5 (23.8%) from 21 patients demonstrated relapses of disseminated encephalomyelitis, and 2 (9.5%) of these 21 patients had multiple sclerosis.…”

“…In the Alan investigations, 13 (33.3%) of 39 patients faced the replaces of demyelination disease [9] [21]. For the long period of observations (8 years), only one patient with ADEM of 11 showed new focuses of demyelination in MRT data [20].…”

Acute Disseminated Encephalomyelitis: A Review of Eleven Cases in Childhood in North of Iran

“…ADEM is more common in winter months and in 80% of childhood cases occurs in the first decade of life (2). The pathogenesis is unclear, but it is thought to be immune-mediated, because in up to threefourths of cases, it follows an antecedent infection or immunization (3). In general, clinical presentation of ADEM may be very heterogeneous.…”

“…In general, clinical presentation of ADEM may be very heterogeneous. However, a combination of altered consciousness or behavior and multifocal neurological deficits, especially in close relation to an infection, should raise clinician's suspicion to consider ADEM in the differential diagnosis (3,4). Some children never have focal neurological signs, whereas in others the initial feature suggests a focal mass lesion (5).…”