ELECTRONIC ARTICLE |
Acute Disseminated Encephalomyelitis in Children
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* Departments of Neurology
Pediatrics, State University of New York at Buffalo, School of Medicine and Biomedical Sciences, Buffalo, New York
Divisions of Child Neurology
|| Infectious Diseases, Childrens Hospital of Buffalo, Buffalo, New York
¶ Imaging Services
# Buffalo Neuroimaging Analysis Center, Jacobs Neurological Institute of Kaleida Health, Buffalo, New York
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.
Key Words: 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
Received for publication Dec 18, 2001; Accepted Apr 8, 2002.
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