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2018 ; 10
(ä): 391-399
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Pediatric acquired demyelinating syndromes: a nationwide validation study of the
Danish National Patient Register
#MMPMID29692631
Boesen MS
; Magyari M
; Born AP
; Thygesen LC
Clin Epidemiol
2018[]; 10
(ä): 391-399
PMID29692631
show ga
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.