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From the Division of Pediatric Neurology (D.J.A.C.), Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Canada; Department of Diagnostic Imaging (M.M.S., H.M.B.), Department of Biostatistics (D.S.), and Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Canada; Department of Radiology (D.K.L., B.L.B.), University of British Columbia, Vancouver, Canada; and Department of Pediatric Neurology (T.L.), Texas Children's Hospital, Baylor College of Medicine, Houston, TX.
Address correspondence and reprint requests to Dr. David J.A. Callen, Department of Pediatric Neurology, McMaster Children's Hospital, 1200 Main St. West, Hamilton, ON, Canada L8N 3Z5 dcallen{at}mcmaster.ca.
Background: Acute disseminated encephalomyelitis (ADEM) is typically a monophasic demyelinating disorder. However, a clinical presentation consistent with ADEM can also be the first manifestation of multiple sclerosis (MS), particularly in children. Quantitative analyses of MRI images from children with monophasic ADEM have yet to be compared with those from children with MS, and MRI criteria capable of distinguishing ADEM from MS at onset have yet to be derived.
Methods: A retrospective analysis of MRI scans obtained at first attack from 28 children subsequently diagnosed with MS and 20 children with ADEM was performed. T2/fluid-attenuated inversion recovery hyperintense lesions were quantified and categorized according to location, description, and size. T1-weighted images before and after administration of gadolinium were evaluated for the presence of black holes and for gadolinium enhancement. Mean lesion counts and qualitative features were compared between groups and analyzed to create a proposed diagnostic model.
Results: Total lesion number did not differentiate ADEM from MS, but periventricular lesions were more frequent in children with MS. Combined quantitative and qualitative analyses led to the following criteria to distinguish MS from ADEM: any two of 1) absence of a diffuse bilateral lesion pattern, 2) presence of black holes, and 3) presence of two or more periventricular lesions. Using these criteria, MS patients at first attack could be distinguished from monophasic ADEM patients with an 81% sensitivity and a 95% specificity.
Conclusions: MRI diagnostic criteria are proposed that may be useful in differentiating children experiencing the first attack of multiple sclerosis from those with monophasic acute disseminated encephalomyelitis.
Abbreviations: ADEM = acute disseminated encephalomyelitis; CIS = clinically isolated syndrome; FLAIR = fluid-attenuated inversion recovery; KIDMUS CC = lesions perpendicular to the long axis of the corpus callosum; KIDMUS discrete = sole presence of well-defined lesions; MS = multiple sclerosis; NA = not applicable; NPV = negative predictive value; ON = optic neuritis; OR = odds ratio; PPV = positive predictive value; RRMS = relapsing–remitting multiple sclerosis; TM = transverse myelitis.
Supplemental data at www.neurology.org.
Editorial, page 952
See also page 961
e-Pub ahead of print on November 26, 2008, at www.neurology.org.
Disclosure: The authors report no disclosures.
Received March 5, 2008. Accepted in final form September 19, 2008.
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