Abstract Details

Title A Systematic Clinico-Serologic Study of Recurrent Longitudinally Extensive Transverse Myelitis (rTM) and Comparison with NMO

Topic MS and Related Diseases

Presentation(s) P02 - (-)

Poster/Presentation
Number 141

Objective

Background BACKGROUND: Seropositive rTM is classified as an NMO-spectrum disorder. Evolving serological assay sensitivity has precluded comparison of the clinical characteristics of rTM and NMO.

Design/Methods DESIGN/METHODS: From 2005-2011, 5349 samples from Mayo Clinic patients were tested by IIF assay. Clinical records revealed 48 patients had rTM (pediatric onset in 3). Of those, 36 (75%) were IIF-positive and 12 (25%) IIF-negative; 11 of the 12 scored negative were available for testing by ELISA (RSR/Kronus) and AQP4-transfected cell-binding assays (CBA slides [EUROIMMUN] and in-house fluorescence-activated-cell-sorting [FACS]). Clinical characteristics were compared based on finally assigned serostatus. 138 AQP4-IgG-positive NMO patients served as control cases.

Results RESULTS: Overall, the AQP4-IgG detection rate improved to 91% by FACS, 85% by CBA, and 83% by ELISA. Only 4 cases remained seronegative: 2 boys (onset ages 3 and 6) and 2 adults (1 male, 1 female). Among 42 seropositive adult rTM cases (excluding single female with onset age 15) the female:male ratio = 5:1. rTM cases required a cane sooner (median 19.5 months) than NMO cases (48 months; p=0.04).Two findings suggest seropositive rTM may progress to NMO: 1) rTM cases had a shorter median disease duration (first attack to last follow-up, 61 months) than control NMO cases (106 months); 2) For the NMO subgroup with initial rTM course, time from onset to 1st optic neuritis (54 months) was similar to rTM cases' median disease duration.

Conclusions CONCLUSIONS: Adult rTM is rarely AQP4-IgG-seronegative. NMO evolution can be anticipated in seropositive cases. The 2 seronegative boys may have MS because [sim] 14% of pediatric MS cases have longitudinally-extensive lesions [Banwell et al, Neurology, 2008]; adult MS cases do not.

Authors/Disclosures
Yujuan Jiao PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
Timothy Cunniff, PharmD	Dr. Cunniff has received personal compensation for serving as an employee of Paragon Biosciences. Dr. Cunniff has stock in Harmony Biosciences. Dr. Cunniff has stock in Emalex Biosciences.
Vanda Lennon, MD, PhD (Mayo Clinic)	The institution of Dr. Lennon has received research support from NIH. Dr. Lennon has received intellectual property interests from a discovery or technology relating to health care.
Andrew McKeon, MD (Mayo Clinic)	The institution of Dr. McKeon has received research support from National Institutes of Health. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received publishing royalties from a publication relating to health care.
	No disclosure on file
	No disclosure on file
Raffaele Iorio, MD (Catholic University)	Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Dianthus Therapeutics. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson & Johnson. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Argenx. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Johnson&Johnson.
Chiara Costanzi	No disclosure on file
Amy M. Quek, MBBS (National University Hospital)	The institution of Dr. Quek has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. The institution of Dr. Quek has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. The institution of Dr. Quek has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Astra Zeneca. The institution of Dr. Quek has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. The institution of Dr. Quek has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Merck. The institution of Dr. Quek has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Astra Zeneca.
Brian G. Weinshenker, MD, FAAN (University of Virginia Health System)	Dr. Weinshenker has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CANbridge Pharmaceuticals. Dr. Weinshenker has received personal compensation in the range of $0-$499 for serving as a Consultant for CALIBR. Dr. Weinshenker has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Horizon. Dr. Weinshenker has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Weinshenker 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 Group (Chugai, Genentech, Roche). Dr. Weinshenker has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB Pharmaceuticals. Dr. Weinshenker has received research support from Guthy Jackson Charitable Foundation. Dr. Weinshenker has received intellectual property interests from a discovery or technology relating to health care.
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.
Elizabeth A. Shuster, MD	No disclosure on file
Claudia F. Lucchinetti, MD, FAAN (University of De Medical School, Health Learning Blg)	The institution of Dr. Lucchinetti has received research support from Biogen Idec. The institution of Dr. Lucchinetti has received research support from NIH/NINDS. The institution of Dr. Lucchinetti has received research support from National Institute of Neurological Disorders and Stroke . The institution of Dr. Lucchinetti has received research support from National Multiple Sclerosis Society. The institution of Dr. Lucchinetti has received research support from National Center for Advancing Translational Sciences. Dr. Lucchinetti has received intellectual property interests from a discovery or technology relating to health care. Dr. Lucchinetti has a non-compensated relationship as a Member with National Institute of Neurological Disorders that is relevant to AAN interests or activities. Dr. Lucchinetti has a non-compensated relationship as a Member with Board of Directors, Association of University Professors of Neurology that is relevant to AAN interests or activities. Dr. Lucchinetti has a non-compensated relationship as a Member with Mayo Clinic Board of Trustees that is relevant to AAN interests or activities. Dr. Lucchinetti has a non-compensated relationship as a Member with Mayo Clinic Board of Governors that is relevant to AAN interests or activities.
Sean J. Pittock, MD, FAAN (Mayo Clinic Dept of Neurology)	Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Pittock 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. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from NIH. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received publishing royalties from a publication relating to health care.

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