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

Journal of Neurology, Mar 2018

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. 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. 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%). 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.

A PDF file should load here. If you do not see its contents the file may be temporarily unavailable at the journal website or you do not have a PDF plug-in installed and enabled in your browser.

Alternatively, you can download the file locally and open with any standalone PDF reader:

https://link.springer.com/content/pdf/10.1007/s00415-018-8835-6.pdf

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

Journal of Neurology Incidence and outcome of acquired demyelinating syndromes in Dutch children: update of a nationwide and prospective study R. F. Neuteboom 0 1 2 3 4 5 6 7 8 9 0 Department of Neurology, Canisius-Wilhelmina Hospital , Nijmegen , The Netherlands 1 Department of Neurology, St. Antonius Hospital , Nieuwegein , The Netherlands 2 Department of Paediatric Neurology, Admiraal de Ruyter Hospital , Goes , The Netherlands 3 Department of Paediatric Neurology , Radboud UMC, Nijmegen , The Netherlands 4 Department of Neurology , Westfriesgasthuis, Hoorn , The Netherlands 5 Department of Neurology, Amphia Hospital , Breda , The Netherlands 6 Department of Neurology, Medical Spectrum Twente , Enschede , The Netherlands 7 Department of Neurology, MS Centre ErasMS, Erasmus MC , Rotterdam , The Netherlands 8 Department of Paediatrics, Rijnstate Hospital , Arnhem , The Netherlands 9 Department of Paediatric Neurology, UMCG , Groningen , The Netherlands 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. Children; Multiple sclerosis; Acquired demyelinating syndromes; Epidemiology; Outcome Introduction Acquired demyelinating syndromes (ADS) are immunemediated demyelinating disorders of the central nervous system (CNS) in children [ 1, 2 ]. ADS encompass a wide spectrum of neurological symptoms depending on the location of inflammation and the severity of demyelination. As the clinical symptoms overlap in this spectrum, international Extended author information available on the last page of the article consensus criteria have been proposed in 2007 to aid in diagnosis and distinction between subtypes [3]. These criteria were revised in 2012 [ 4 ]. In addition, new findings in the past few years added valuable insights into paediatric ADS and its subtypes, including the identification of new biomarkers such as anti-myelin oligodendrocyte glycoprotein antibodies (MOG-ab) [ 2, 5 ] and the identification of new clinical subtypes as acute disseminated encephalomyelitis followed by optic neuritis (ADEM-ON) [6]. ADS may remain monophasic after the first event. Yet, 15–32% of these children will fulfil the diagnostic criteria for paediatric MS within 5 years after the initial attack [ 1, 2, 7, 8 ]. Multiple aspects of outcome of paediatric MS patients have been described before, including the rate of disease progression in Expanded Disability Status Scale (EDSS) scores [ 9, 10 ], cognitive performance [ 11–13 ], decreased motor performance [ 14, 15 ], and neuropsychiatric complaints like fatigue and mood disorders [ 14, 16 ]. However, studies describing the long-term outcome of other ADS subtypes are scarce. In the Netherlands, a multicentre and prospective study was established in 2006 with national coverage for children with a first demyelinating event. Incidence estimates of paediatric ADS and multiple sclerosis have been reported in our prior work for the period between 2007 and 2010 [ 17 ]. However, the number of patients who will be diagnosed with MS will likely increase with longer follow-up time. Furthermore, an increasing MS incidence in children has been reported in specific regions [ 18, 19 ]. Therefore, we aim to re-assess the incidence and presenting characteristics of ADS and its subtypes in the Netherlands. Second, we aim to provide long-term follow-up data of the patien (...truncated)


This is a preview of a remote PDF: https://link.springer.com/content/pdf/10.1007/s00415-018-8835-6.pdf

de Mol, C. L., Wong, Y. Y. M., van Pelt, E. D., Ketelslegers, I. A., Bakker, D. P., Boon, M., Braun, K. P. J., van Dijk, K. G. J., Eikelenboom, M. J., Engelen, M., Geleijns, K., Haaxma, C. A., Niermeijer, J. M. F., Niks, E. H., Peeters, E. A. J., Peeters-Scholte, C. M. P. C. D., Poll-The, B. T., Portier, R. P., de Rijk-van Andel, J. F., Samijn, J. P. A., Schippers, H. M., Snoeck, I. N., Stroink, H., Vermeulen, R. J., Verrips, A., Visscher, F., Vles, J. S. H., Willemsen, M. A. A. P., Catsman-Berrevoets, C. E., Hintzen, R. Q., Neuteboom, R. F.. Incidence and outcome of acquired demyelinating syndromes in Dutch children: update of a nationwide and prospective study, Journal of Neurology, 2018, pp. 1310-1319, Volume 265, Issue 6, DOI: 10.1007/s00415-018-8835-6