Abstract
Background: ADEM, although relatively uncommon, is probably under-recognized. Objectives: To spotlight on the clinical profile and therapeutic outcome of children with ADEM. Methods: This is a prospective study of patients with ADEM who were admitted to the Pediatric Departments in Aladan and Alfarawanya Hospitals in Kuwait, from January 2009 to January 2011. Clinical, microbiological, radiological and therapeutic data were analyzed. Results: Of 48 patients presented with acute neurological symptoms and signs, 21 patients fulfilled criteria for ADEM. 80.95% of cases were presenting in winter and spring, 57% of patients had a history of upper respiratory tract illness. The commonest presentations were motor deficits, convulsions followed by altered conscious. CSF virology studies showed Herpes Simplex Virus (HSV) and Epstein-Barr virus (EBV) in 3 patients whereas nasal and nasopharyngeal swab showed evidence of influenza H1N1 virus in 1 patient. Brain MRI was performed in all patients and revealed multiple hyperintense supratentorial brain lesions on T2/FLAIR images. 85.7% of patients had cortical and/or subcortical white matter lesions which were bilateral and asymmetric in location and size. Most of the patients were treated with steroids alone, 3 patients received high-dose intravenous methylprednisolone and 6 patients received both steroids and intravenous immunoglobulin.
Highlights
Of 48 patients presented with acute neurological symptoms and signs, 21 patients fulfilled criteria for Acute Demyelinating Encephalomyelitis (ADEM). 80.95% of cases were presenting in winter and spring, 57% of patients had a history of upper respiratory tract illness
Brain magnetic resonance imaging (MRI) was performed in all patients and revealed multiple hyperintense supratentorial brain lesions on T2/FLAIR images. 85.7% of patients had cortical and/or subcortical white matter lesions which were bilateral and asymmetric in location and size
Acute Disseminated Encephalomyelitis (ADEM) is a monophasic acute nonvasculitic inflammatory demyelinating disorder of the central nervous system characterized by diffuse neurologic signs and symptoms coupled with evidence of multifocal lesions of demyelination on neuroimaging
Summary
Acute Disseminated Encephalomyelitis (ADEM) is a monophasic acute nonvasculitic inflammatory demyelinating disorder of the central nervous system characterized by diffuse neurologic signs and symptoms coupled with evidence of multifocal lesions of demyelination on neuroimaging. It is first described in the early 18th century as uncommon presentation of measles and small pox. McAlpine in 1931 [3] described 3 sets of patients with ADEM: 1) postvaccination, 2) after infectious fevers and 3) spontaneous. A number of recent reports of ADEM in children have confirmed the observations of McAlpine [4,5].
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