A comparative evaluation of different neuromyelitis optica spectrum disorder sets of criteria.

C Papeix,C Lubetzki,E Maillart,R Marignier,S Vukusic,N Collongues,J De Seze,Y Beigneux

doi:10.1111/ene.14414

Abstract

Three different sets of criteria have been proposed for the diagnosis of neuromyelitis optica spectrum disorder (NMOSD). The objective was to compare the specificity, sensitivity and diagnostic accuracy of the three different sets of NMOSD criteria in patients presenting with inflammatory disorders of the central nervous system suggestive of NMOSD. For 236 suspected NMOSD patients referred for serum aquaporin-4 immunoglobulin G antibody (AQP4-IgG) testing between 2012 and 2014, the three sets of NMOSD criteria [1999, 2006 NMO criteria and 2015 International Panel for NMO Diagnosis (IPND) criteria] were applied and compared to the final diagnosis. Seventy-six patients fulfilled at least one set of criteria and 28 patients fulfilled all NMOSD sets of criteria. The final diagnosis was NMOSD in 66 cases, multiple sclerosis according to the MacDonald 2010 criteria in 85 cases and another diagnosis in 85 cases. The 2006 NMO criteria have the highest specificity (99%) and the 2015 IPND NMOSD criteria the highest sensitivity (97%). For the 1999, 2006 and 2015 IPND NMOSD criteria, the accuracy was respectively 82%, 87% and 97%. Our study highlights the limitations of the first set of criteria that include the optico-spinal form of multiple sclerosis. The accuracy of NMOSD diagnostic criteria improved from 1999 to 2015. It confirms the increased performance of the last set of criteria which covers a larger spectrum of clinical presentation. This study raises some concerns for classifying patients with seronegative transverse myelitis or optic neuritis, and myelin oligodendrocyte glycoprotein (MOG) antibody- associated disease.

Highlights

Since 1999, three different sets of diagnostic criteria have been proposed, mainly to distinguish NMO from multiple sclerosis (MS) and other CNS inflammatory disorders
From 236 suspected neuromyelitis optica spectrum disorders (NMOSD) referred for serum AQP4-IgG testing between 2012 and 2014, the three sets of NMOSD criteria (1999, 2006 NMO criteria and 2015 International Panel for NMO Diagnosis criteria) were applied and compared to the final diagnosis
Our study highlights the limitations of the first set of criteria, that include optico-spinal form of MS

Summary

Introduction

Since 1999, three different sets of diagnostic criteria have been proposed, mainly to distinguish NMO from multiple sclerosis (MS) and other CNS inflammatory disorders. NMO diagnosis was based essentially on a probabilistic combination of clinical signs, brain or spinal cord MRI and CSF analysis.[1] The identification of aquaporin-4 immunoglobulin G antibody (AQP4-IgG)[2] led to redesign diagnostic criteria in 2006.3 In 2015, the International Panel for NMO Diagnosis (IPND) revised the diagnostic criteria for NMO/SD4, taking into account the broadening clinical spectrum. This new set of criteria allowed an earlier diagnosis by the inclusion of positive AQP4-IgG (AQP4-IgG +) clinically limited forms, and patients with lesions outside the optic nerve and the spinal cord. Three different sets of criteria have been proposed for the diagnosis of neuromyelitis optica spectrum disorders (NMOSD)

Methods

Results

Conclusion