title: Incidence of acquired demyelinating syndromes of the CNS in Dutch children: a nationwide study.
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.
authors.affiliation: MS Centre ErasMS, Department of Neurology, Room Ee 2230, Erasmus MC, PO Box 2040, 3000 CA, Rotterdam, The Netherlands. i.ketelslegers@erasmusmc.nl
authors.forename: I A
authors.initials: IA
authors.lastname: Ketelslegers
authors.forename: C E
authors.initials: CE
authors.lastname: Catsman-Berrevoets
authors.forename: R F
authors.initials: RF
authors.lastname: Neuteboom
authors.forename: M
authors.initials: M
authors.lastname: Boon
authors.forename: K G J
authors.initials: KG
authors.lastname: van Dijk
authors.forename: M J
authors.initials: MJ
authors.lastname: Eikelenboom
authors.forename: R H J M
authors.initials: RH
authors.lastname: Gooskens
authors.forename: E H
authors.initials: EH
authors.lastname: Niks
authors.forename: W C G
authors.initials: WC
authors.lastname: Overweg-Plandsoen
authors.forename: E A J
authors.initials: EA
authors.lastname: Peeters
authors.forename: C M P C D
authors.initials: CM
authors.lastname: Peeters-Scholte
authors.forename: B T
authors.initials: BT
authors.lastname: Poll-The
authors.forename: J F
authors.initials: JF
authors.lastname: de Rijk-van Andel
authors.forename: J P A
authors.initials: JP
authors.lastname: Samijn
authors.forename: I N
authors.initials: IN
authors.lastname: Snoeck
authors.forename: H
authors.initials: H
authors.lastname: Stroink
authors.forename: R J
authors.initials: RJ
authors.lastname: Vermeulen
authors.forename: A
authors.initials: A
authors.lastname: Verrips
authors.forename: J S H
authors.initials: JS
authors.lastname: Vles
authors.forename: M A A P
authors.initials: MA
authors.lastname: Willemsen
authors.forename: R
authors.initials: R
authors.lastname: Rodrigues Pereira
authors.forename: R Q
authors.initials: RQ
authors.lastname: Hintzen
mesh_terms: D000293:Adolescent; D002648:Child; D020278:Demyelinating Autoimmune Diseases, CNS / Q000134:cerebrospinal fluid / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology*; D005260:Female; D006801:Humans; D015994:Incidence; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D010372:Pediatrics; D012189:Retrospective Studies; D018709:Statistics, Nonparametric