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Observational Study
. 2018 Jun;265(6):1310-1319.
doi: 10.1007/s00415-018-8835-6. Epub 2018 Mar 22.

Incidence and outcome of acquired demyelinating syndromes in Dutch children: update of a nationwide and prospective study

C L de Mol  1 , Y Y M Wong  1 , E D van Pelt  1 , I A Ketelslegers  1 , D P Bakker  2 , M Boon  3 , K P J Braun  4 , K G J van Dijk  5 , M J Eikelenboom  6 , M Engelen  7 , K Geleijns  4 , C A Haaxma  8 , J M F Niermeijer  9 , E H Niks  10 , E A J Peeters  11 , C M P C D Peeters-Scholte  10 , B T Poll-The  7 , R P Portier  12 , J F de Rijk-van Andel  13 , J P A Samijn  14 , H M Schippers  15 , I N Snoeck  11 , H Stroink  16 , R J Vermeulen  17 , A Verrips  16 , F Visscher  18 , J S H Vles  17 , M A A P Willemsen  8 , C E Catsman-Berrevoets  19 , R Q Hintzen  1 , R F Neuteboom  20
Affiliations
Observational Study

Incidence and outcome of acquired demyelinating syndromes in Dutch children: update of a nationwide and prospective study

C L de Mol et al. J Neurol. 2018 Jun.

Abstract

Introduction: Acquired demyelinating syndromes (ADS) are immune-mediated demyelinating disorders of the central nervous system in children. A nationwide, multicentre and prospective cohort study was initiated in the Netherlands in 2006, with a reported ADS incidence of 0.66/100,000 per year and MS incidence of 0.15/100,000 per year in the period between 2007 and 2010. In this study, we provide an update on the incidence and the long-term follow-up of ADS in the Netherlands.

Methods: Children < 18 years with a first attack of demyelination were included consecutively from January 2006 to December 2016. Diagnoses were based on the International Paediatric MS study group consensus criteria. Outcome data were collected by neurological and neuropsychological assessments, and telephone call assessments.

Results: Between 2011 and 2016, 55/165 of the ADS patients were diagnosed with MS (33%). This resulted in an increased ADS and MS incidence of 0.80/100,000 per year and 0.26/100,000 per year, respectively. Since 2006 a total of 243 ADS patients have been included. During follow-up (median 55 months, IQR 28-84), 137 patients were diagnosed with monophasic disease (56%), 89 with MS (37%) and 17 with multiphasic disease other than MS (7%). At least one form of residual deficit including cognitive impairment was observed in 69% of all ADS patients, even in monophasic ADS. An Expanded Disability Status Scale score of ≥ 5.5 was reached in 3/89 MS patients (3%).

Conclusion: The reported incidence of ADS in Dutch children has increased since 2010. Residual deficits are common in this group, even in monophasic patients. Therefore, long-term follow-up in ADS patients is warranted.

Keywords: Acquired demyelinating syndromes; Children; Epidemiology; Multiple sclerosis; Outcome.

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Conflict of interest statement

Conflicts of interest

C. L. de Mol, Y. Y. M. Wong, E. D. van Pelt, I. A. Ketelslegers, D. P. Bakker, M. Boon, K. P. J. Braun, K. G. J. van Dijk, M. J. Eikelenboom, M. Engelen, K. Geleijns, C. A. Haaxma, J. M. F. Niermeijer, E. H. Niks, E. A. J. Peeters, C. M. P. C. D. Peeters-Scholte, B. T. Poll-The, R. P. Portier, J. F. de Rijk-van Andel, J. P. A. Samijn, H. M. Schippers, I. N. Snoeck, H. Stroink, R. J. Vermeulen, A. Verrips, F. Visscher, J. S. H. Vles, M. A. A. P. Willemsen and C. E. Catsman-Berrevoets report no conflict of interest in respect to this work. R. Q. Hintzen received honoraria for serving on advisory boards for Biogen Idec, Roche, Sanofi. He participated in trials with BiogenIdec, Merck-Serono, Roche, Genzyme and Novartis. R. F. Neuteboom participates in trials with Sanofi Genzyme and Novartis.

Ethical standards

This study was approved by the Medical Ethical Committees of the Erasmus MC in Rotterdam and the other participating centres.

Figures

Fig. 1
Fig. 1
Incidence of acquired demyelinating syndromes (ADS) and multiple sclerosis (MS) in the Netherlands in 2007–2010 and 2011–2016
Fig. 2
Fig. 2
Flowchart describing the selection process
Fig. 3
Fig. 3
Distribution of clinical subtypes of patients diagnosed with multiphasic non-MS (n = 17). ADEM acute disseminated encephalomyelitis, ON optic neuritis, NMOSD neuromyelitis optica spectrum disorder, MDEM multiphasic acute disseminated encephalomyelitis
Fig. 4
Fig. 4
Residual deficits of ADS patients at last follow-up. Definition: patients were classified as cognitive impaired if they had a deficit in at least one cognitive domain, tested by a neuropsychological assessment
See this image and copyright information in PMC

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