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. 2012 Sep;259(9):1929-35.
doi: 10.1007/s00415-012-6441-6. Epub 2012 Feb 17.

Incidence of acquired demyelinating syndromes of the CNS in Dutch children: a nationwide study

I A Ketelslegers  1 , C E Catsman-Berrevoets, R F Neuteboom, M Boon, K G J van Dijk, M J Eikelenboom, R H J M Gooskens, E H Niks, W C G Overweg-Plandsoen, E A J Peeters, C M P C D Peeters-Scholte, B T Poll-The, J F de Rijk-van Andel, J P A Samijn, I N Snoeck, H Stroink, R J Vermeulen, A Verrips, J S H Vles, M A A P Willemsen, R Rodrigues Pereira, R Q Hintzen
Affiliations

Incidence of acquired demyelinating syndromes of the CNS in Dutch children: a nationwide study

I A Ketelslegers et al. J Neurol. 2012 Sep.

Abstract

Acquired demyelinating syndromes (ADS) can be a first presentation of multiple sclerosis (MS) in children. The incidence of these disorders in Europe is currently unknown. Children (<18 years old) living in the Netherlands who presented with ADS were included from January 1, 2007 to December 31, 2010 by the Dutch pediatric MS study group and the Dutch surveillance of rare pediatric disorders. Demographic and clinical data were collected. Eighty-six patients were identified over 4 years, resulting in an incidence of 0.66/1,00,000 per year. Most patients presented with polyfocal ADS without encephalopathy (30%), followed by polyfocal ADS with encephalopathy (24%), optic neuritis (ON, 22%), monofocal ADS (16%), transverse myelitis (3%), and neuromyelitis optica (3%). Patients with polyfocal ADS with encephalopathy were younger (median 3.9 years) than patients with ON (median 14.6 years, p < 0.001) or monofocal ADS (median 16.0 years, p < 0.001). Patients with polyfocal ADS without encephalopathy (median 9.2 years) were also younger than monofocal ADS patients (median 16.0 years, p < 0.001). There was a slight female preponderance in all groups except the ON group, and a relatively large number of ADS patients (29%) reported a non-European ancestry. Familial autoimmune diseases were reported in 23%, more often in patients with relapsing disease than monophasic disease (46 vs. 15%, p = 0.002) and occurring most often in the maternal family (84%, p < 0.001). During the study period, 23% of patients were subsequently diagnosed with MS. The annual incidence of ADS in the Netherlands is 0.66/1,00,000 children/year. A polyfocal disease onset of ADS was most common.

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Figures

Fig. 1
Fig. 1
Age distribution of the patients, categorized by clinical presentation. The median age per group is shown. The horizontal lines above the groups indicate statistical differences between the groups (Mann–Whitney U test, *** p < 0.001). ON optic neuritis, TM transverse myelitis, ADS acquired demyelinating syndrome, mono monofocal, poly polyfocal NMO neuromyelitis optica
See this image and copyright information in PMC

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