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Pediatric acquired demyelinating syndromes: a nationwide validation study of the Danish National Patient Register

Authors Boesen MS, Magyari M, Born AP, Thygesen LC

Received 14 November 2017

Accepted for publication 15 January 2018

Published 10 April 2018 Volume 2018:10 Pages 391—399


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 3

Editor who approved publication: Professor Vera Ehrenstein

Magnus Spangsberg Boesen,1 Melinda Magyari,2,3 Alfred Peter Born,1 Lau Caspar Thygesen4

1Department of Pediatrics, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark; 2The Danish Multiple Sclerosis Registry, Department of Neurology, Rigshospitalet, University of Copenhagen, Denmark; 3Danish Multiple Sclerosis Center, Department of Neurology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark; 4National Institute of Public Health, University of Southern Denmark, Copenhagen, Denmark

Objective: To validate the Danish National Patient Register’s (NPR) diagnoses of pediatric acquired demyelinating syndromes (ADS) including multiple sclerosis (MS).
Study design and setting:
We identified ADS diagnostic groups using International Classification of Diseases (ICD) codes and reviewed medical records to validate the NPR diagnoses during 2008–2015.
Results: Among 409 children in the study, 184 children had a validated and final ADS diagnosis after reviewing medical records as follows: optic neuritis (ON; n=46), transverse myelitis (TM; n=16), acute disseminated encephalomyelitis (ADEM; n=50), clinically isolated syndrome (CIS) including dissemination in space (CIS [DIS]) but not dissemination in time (n=6), neuromyelitis optica spectrum disorder (NMOsd; n=5), and MS (n=61). During the mean follow-up of 4.6 years, 33% of children initially diagnosed with monophasic ADS progressed to MS. Positive predictive value (PPV) was 0.71 (95% confidence interval [CI] =0.62–0.80) for ON, 0.64 (95% CI =0.43–0.82) for TM, 0.93 (95% CI =0.84–0.98) for MS, 0.27 (95% CI =0.19–0.35) for CIS, 0.43 (95% CI =0.10–0.82) for NMOsd, and 0.15 (95% CI =0.10–0.20) for ADEM. Assuming complete coverage for non-MS ADS, the sensitivity was 0.99 (95% CI =0.93–1.00) for ON, 0.83 (95% CI =0.36–1.00) for CIS (DIS), and 0.80 (95% CI =0.56–0.94) for TM, but only 0.58 (95% CI =0.43–0.72) for ADEM and 0.60 (95% CI =0.15–0.95) for NMOsd.
Conclusion: PPV was high for MS and considered acceptable for ON and TM; therefore, these ICD revision 10 (ICD-10) codes from the NPR are useful for epidemiological studies. Conversely, PPV was low for CIS and ADEM; NMOsd was inconclusive.

Keywords: pediatric MS, acquired demyelinating syndrome, ADEM, health registers, validation, ICD-10

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