Comparative evaluation of successive criteria for neuromyelitis optica
ECTRIMS Online Library. Marignier R. Oct 26, 2017; 202452
Dr. Romain Marignier
Dr. Romain Marignier
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Abstract: 97

Type: Oral

Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis

Background: There is currently no gold standard for neuromyelitis optica (NMO) diagnosis. During the past 20 years, 3 main sets of diagnostic criteria have been proposed. The more recent ones (IPND 2015) are considered to improve the diagnostic yield. However, specificity has not been fully evaluated, neither direct comparison between these 3 successive sets of criteria.
Aim: To evaluate the diagnostic accuracy of 3 different sets of NMO criteria, among patients suspected being part of the NMO spectrum disorders.
Methods: We retrospectively evaluated patients referred by three French tertiary centres, for serum AQP4-IgG assay, during the period 2012-2014. We selected patients with complete ascertained clinical, imaging and biological data and for whom a clear diagnosis was finally available. We then apply three different sets of NMO criteria (Wingerchuk 1999, Wingerchuk 2006 and IPND 2015). To evaluate the specificity, we also applied multiple sclerosis (MS) criteria (imaging and clinical, Polman 2015).
Results: Two hundred and thirty five patients were finally eligible for analyses. 75 fulfilled NMO criteria in one or more sets, 76 MS criteria and 94 did not satisfied any criteria. Among the 75 NMO patients, 69/75 (92%) were identified by the 2015 criteria, 38/75(51%) by the 2006 ones and 35/75 (46%) by the 1999 ones. Considering cases identified only by one set of criteria, 29 were detected only by the IPND 2015, none by the 2006 ones, but 6 by the 1999 criteria only. Interestingly 10 patients fulfilling NMO criteria (9/10 fulfilling the IPND 2015, 1/10 the 1999 ones) also satisfied MS criteria, 8 were AQP4-Ab positive. Among the 94 patients not fulfilling any criteria, 46 presented as isolated or recurrent inflammatory optic neuritis/transverse myelitis, associated with negative brain MRI. Finally, among 42 patients tested positive for AQP4-Ab, 41 were considered NMO and one as atypical progressive multiple sclerosis.
Conclusion: IPND 2015 criteria confirmed a higher sensitivity but were not able to catch all the cases previously identified. In addition, our study pointed out an overlap between MS and NMO criteria for some patients. Finally, a large group of patients presenting with NMO related phenotype does not currently fulfill any acknowledged criteria.
R Marignier serves on scientific advisory board for MedImmune and has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva
C Papeix: nothing to disclose
E Maillart reports participation to meetings and advisory boards for Biogen, Genzyme, Merck, Novartis, Roche and Teva, with no relation to the submitted work
J De Seze: nothing to disclose
S Vukusic has received consultancy fees, speaker fees, research grants (non-personal) or honoraria from Biogen, Geneuro, Genzyme-Sanofi, Medday, Merck-Serono, Novartis, Roche and Teva.
N Collongues has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva, with no relation to this study

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