New Acute Severity Scale for Neuromyelitis Optica Relapses (P5.249)

OBJECTIVE: To develop and validate an objective scale that represents degree of neurological dysfunction from an acute relapse of neuromyelitis optica. BACKGROUND: Neuromyelitis optica (NMO) is an autoimmune disease of the central nervous system (CNS) distinct from multiple sclerosis (MS). It is characterized by recurrent episodes of optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM), which commonly lead to incremental event-related disability. Disability generally has been rated using the Expanded Disability Severity Scale (EDSS) developed for MS trials. However, the EDSS is insensitive to changes in visual acuity and limited in patients who cannot walk. DESIGN/METHODS: We designed a 34-point acute relapse severity scale specific to NMO, named the NMO Severity Scale (NMOSS) focusing on areas most significantly affected including 1) Visual Acuity, 2) Visual Fields, 3) Motor, 4) Sensory, 5) Bowel & Bladder, and 6) Brainstem Functions. We asked 17 international experts in NMO to review records from 20 acute NMO admissions to the Johns Hopkins Hospital to evaluate inter-rater reliability and accuracy of the NMOSS. RESULTS: The NMOSS is based on the EDSS but was modified to increase the sensitivity to visual acuity and visual fields. Motor, sensory and bowel/bladder scores were only slightly modified and the brainstem score was changed to focus on NMO-specific attacks of the area postrema and diencephalon. Validation of the NMOSS is ongoing; inter-rater reliability will be calculated using Spearman9s rank correlation coefficient and the NMOSS will be analyzed for content and predictive validity. CONCLUSIONS: The NMOSS is a new NMO-specific disability scale for future clinical trials. Study Supported by: The Guthy Jackson Charitable Foundation Disclosure: Acorda Therapeutics; Sanofi; Viropharma, Inc.; NeuralStem, Inc.; Shire/Abvie; TerumoBCT Dr. Mealy has nothing to disclose. Dr. Jarius has received research support from Bayer Healthcare and Merck Serono. Dr. Paul has received personal compensation for activities with Teva Neuroscience, Sanofi-Aventis Pharmaceuticals, Bayer Schering Pharma, Merck Serono, Biogen Idec, MedImmune, and Novartis., Dr. Aktas has received personal compensation for activities with Bayer, Biogen Idec, Genzyme, Novartis, Teva Neuroscience, and Medimmune as an advisor. Dr. Broadley has nothing to disclose. Dr. Cabre has received personal compensation for activities with Biogen Idec, EMD Serono, and Novartis. Dr. Han has nothing to disclose. Dr. Jacob has nothing to disclose. Dr. John has received research support from Teva. Dr. Leite has nothing to disclose. Dr. Marignier has nothing to disclose. Dr. Nakashima has received personal compensation for activities with Bayer Schering Pharma, Novartis, and Biogen Idec. Dr. Nakashima has received research support from Mitsubishi Chemical Corporation. Dr. Palace has received personal compensation for activities with Novartis and Merck Serono. Dr. Schippling has received personal compensation for activities with Novartis. Dr. Schippling has received research support from Bayer Schering and Biogen Idec. Dr. Traboulsee has received personal compensation for activities with Roche Diagnostics Corporation, EMD Serono, Teva Neuroscience, and Biogen Idec, Novartis, and Genzyme Corporation. Dr. Weinshenker has received personal compensation for activities with Novartis, Biogen Idec, and Mitsubishi Pharmaceuticals, MedImmune Pharmaceuticals, Alexion Pharmaceuticals, Chugai Pharmaceutical, and Novartis. Dr. Weinshenker has received royalty pay Dr. Wingerchuk has received personal compensation for activities with Medimmune, Alexion, and Chugai. Dr. Wingerchuk has received research support from Alexion and TerumoBCT.