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Published online before print July 30, 2008, doi:10.1212/01.wnl.0000316193.89691.e1)
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 Volume 71, Number 13, September 23, 2008
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NEUROLOGY 2008;71:967-973
© 2008 American Academy of Neurology

Prognostic factors after a first attack of inflammatory CNS demyelination in children

R. F. Neuteboom, MD, M. Boon, MD, PhD, C. E. Catsman Berrevoets, MD, PhD, J. S. Vles, MD, PhD, R. H. Gooskens, MD, PhD, H. Stroink, MD, R. J. Vermeulen, MD, PhD, J. J. Rotteveel, MD, PhD, I. A. Ketelslegers, MD, E. Peeters, MD, B. T. Poll-The, MD, PhD, J. F. De Rijk-Van Andel, MD, PhD, A. Verrips, MD, PhD and R. Q. Hintzen, MD, PhD

From the Department of Neurology (R.F.N., C.E.C.B., I.A.K., R.Q.H.), Erasmus MC, Rotterdam; Department of Neurology (M.B.), University Hospital, Groningen; Department of Neurology (J.S.V.), University Hospital, Maastricht; Department of Neurology (R.H.G.), University Hospital, Utrecht; Department of Neurology (H.S.), St. Elisabeth Hospital and TweeSteden Hospital, Tilburg; Department of Neurology (R.J.V.), VUMC, Amsterdam; Department of Pediatric Neurology (J.J.R.), Radboud University Medical Centre, Nijmegen; Department of Neurology (E.P.), Juliana Children's Hospital, The Hague; Department of Pediatric Neurology (B.T.P-T.), Emma Children's Hospital/AMC, Amsterdam; Department of Neurology (J.F.D.R.-V.A.), Amphia Hospital, Breda; and Department of Neurology (A.V.), Canisius Hospital Nijmegen, The Netherlands.

Address correspondence and reprint requests to Dr Hintzen, Department of Neurology, MS centre ErasMS, Erasmus MC r.hintzen{at}erasmusmc.nl

Objective: To identify clinical, radiologic, or CSF factors that predict conversion to multiple sclerosis (MS) after a first attack of inflammatory demyelination in children.

Methods: In this nationwide retrospective multicenter study in the Netherlands, 117 children below age 16 were included. Fifty-four children presented with a monofocal clinically isolated syndrome (CIS) and 63 children with a polyfocal CIS (PCIS).

Results: A second MS-defining attack occurred in 43% of the CIS cases, compared to 21% of the patients with PCIS onset (p < 0.006). Basal ganglia and thalamic lesions and lesions larger than 2 cm on MRI (considered typical of ADEM) were observed during PCIS, irrespective of the presence of encephalopathy. No significant difference in developing MS was found in children with PCIS with or without encephalopathy. Elevated IgG index and presence of oligoclonal CSF bands were more often observed in children who developed MS. Both Barkhof and KIDMUS MRI criteria shared a high specificity and had a high positive predictive value for conversion to MS. In children under the age of 10, the Barkhof criteria had a higher sensitivity than the KIDMUS criteria, but still lower than in older children.

Conclusions: Barkhof and KIDMUS MRI criteria share a high specificity and positive prognostic value for conversion to multiple sclerosis (MS). Sensitivity of these criteria is poor, especially in children below 10 years of age. Basal ganglia lesions can occur in patients who later develop MS. A substantial number of patients presenting with polyfocal onset and no encephalopathy remained monophasic.

Abbreviations: ADEM = acute disseminated encephalomyelitis; CIS = clinically isolated syndrome; MS = multiple sclerosis; OCB = oligoclonal bands; PCIS = polyfocal CIS.

Editorial, page 962.

e-Pub ahead of print on July 30, 2008, at www.neurology.org.

MS Center ErasMS received financial support for this study from the Netherlands MS Research Foundation. R.Q.H. is supported by a grant from the Dutch Society for Research NWO.

Disclosure: The authors report no disclosures.

Received October 2, 2007. Accepted in final form February 29, 2008.




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