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

Abstract

IntroductionAcquired 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.MethodsChildren < 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.ResultsBetween 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%).ConclusionThe 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.

Pediatric autoimmune encephalitis

“…Autoimmune encephalitis (AIE) has expanded the already comprehensive list of pediatric neuroinflammatory disorders of the CNS. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and acute disseminated encephalomyelitis (ADEM) are the most frequently described cause of AIE in children, [1][2][3][4] and disease courses have been studied in detail, including treatment responses, functional recovery, 1,4 and long-term neuropsychological outcome. 5 Next to anti-NMDAR, other neuronal antibodies have been described only sporadically in children, [6][7][8] whereas in adults, reported incidence of these antibodies has increased dramatically.…”

“…This category consisted partially of children with clinically defined acquired AE/inflammatory disorders, such as Rasmussen encephalitis or Sydenham chorea, and partially of children with MRI or CSF abnormalities pointing toward an AE/inflammatory etiology (pleocytosis, elevated protein, or oligoclonal bands in CSF, and MRI lesions in the temporal lobe), with exclusion of other causes, and not fulfilling the criteria of seronegative AIE. 11 (4) Unknown etiology and no support for AE/inflammatory, including children without MRI or CSF abnormalities pointing toward and AE/inflammatory etiology. 5…”

Multiple Sclerosis in Children: Current and Emerging Concepts

“…Studies have reported an incidence of MS in children between 0.26 and 2.1 per 100,000 children, depending on the geographic location. [6][7][8][9][10][11] Some of these studies have reported a rising incidence of MS in children; however, improved awareness of the diagnosis and changes in diagnostic criteria likely impact these estimates.…”

Effects of the Positive Threshold and Data Analysis on Human MOG Antibody Detection by Live Flow Cytometry

“…In brief, patient serum (1:50) was incubated with a transduced cell line expressing full-length human MOG, followed by fluorochromeconjugated anti-human IgG (H+L). Dilution of serum at 1:50 was standard and was most frequently used across studies (2,3,6,8,23,24,30,32,38) Table 2). Samples were reported positive if they were above the positive threshold in at least two of three quality-controlled experiments, a feature that may not have been implemented in other studies but ensures a reliable serostatus report and provides insight into serostatus reproducibility (21).…”