Abstract Details

Title Incidence and Clinical Characteristics of Secondary Progressive Neuromyelitis Optica

Topic MS and Related Diseases

Presentation(s) P02 - (-)

Poster/Presentation
Number 149

Objective

Background BACKGROUND: Secondary progressive (SP) disease underlies development of unrelenting disability in multiple sclerosis (MS). In contrast, disability in NMO is related to residua of severe attacks and SP-NMO appears uncommon. Because little is known about SP-NMO, we re-evaluated its incidence and studied its phenotypic characteristics.

Design/Methods DESIGN/METHODS: We reviewed the course of 116 NMO patients (defined by Wingerchuk et al, Neurology, 2006) evaluated at Mayo Clinic Arizona. SP-NMO was defined as gradual, unremitting neurological deterioration for at least one year. Subjects had at least two limbs with evaluable motor strength and one eye with evaluable vision. Confirmed progression required at least one of: a) increase of at least 2 points in NMO visual acuity score (0-7 scale); b) strength deterioration in one or more limbs in at least two antigravity muscles; c) an increase of 2 or more EDSS points. Demographic, clinical and imaging characteristics were recorded for SP-NMO patients from this cohort and a prior study.

Results RESULTS: SP-NMO occurred in 2 subjects (incidence 1.7%). Onset age was 30-38 y (mean 34y) and all were NMO-IgG seropositive. The number of relapses (range 1-10) and disease duration (range 3-20y) prior to SP onset was highly variable. All received immunosuppressive treatment prior to SP onset. EDSS at SP onset ranged from 3.0-6.5 (mean 5.2), and final EDSS was 6.5-8.5 (mean 7.2). SP-NMO manifested as progressive cervical myelopathy, but insidious-onset visual progression also occurred in 2/3 patients.

Conclusions CONCLUSIONS: SP-NMO is uncommon, appears to occur in NMO-IgG patients, and can be clinically expressed in CNS regions both affected and unaffected by previous clinical relapses. The implications for understanding pathophysiological mechanisms of SP disease in both MS and NMO are discussed.

Authors/Disclosures
Melissa M. Cortez, DO (University of Utah Neurology) PRESENTER	The institution of Dr. Cortez has received research support from NIH NINDS. The institution of Dr. Cortez has received research support from Dysautonomia International. Dr. Cortez has received personal compensation in the range of $500-$4,999 for serving as a Content Expert, Reviewer, DSMB with NIH RECOVER project.
Oscar L. Lopez, MD, FAAN	Dr. Lopez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Acumen.
Dean M. Wingerchuk, MD, FAAN (Mayo Clinic)	Dr. Wingerchuk has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Dr. Wingerchuk has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Wingerchuk has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Wingerchuk has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Wingerchuk has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Meyer Squibb. Dr. Wingerchuk has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB Pharma. Dr. Wingerchuk has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca. Dr. Wingerchuk has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Wingerchuk has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abcuro. Dr. Wingerchuk has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wolters Kluwers.

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