Abstract Details

Title Myelin Oligodendrocyte Glycoprotein Autoantibody Myelitis; Clinical characteristics, MRI clues and discriminators from other demyelinating myelitis etiologies

Topic Autoimmune Neurology

Presentation(s) S21 - Autoimmune Neurology: Novel Diagnostic and Predictive Biomarkers and Immunopathologic Mechanisms of Disease (4:14 PM-4:25 PM)

Poster/Presentation
Number 005

Objective

To evaluate the clinical, radiological and prognostic features of MOG-IgG myelitis and compare it to Aquaporin 4 (AQP4)-IgG and multiple sclerosis (MS) myelitis.

Background Accumulating evidence has suggested that detection of MOG-IgG defines a clinical syndrome distinct from both MS and AQP4-IgG seropositive NMOSD. Recognition of clinical and radiologic features of MOG-IgG myelitis could allow earlier diagnosis and thereby improve prognostication and treatment decisions.

Design/Methods

Mayo Clinic patients (2000-2017) with myelitis and seropositivity for MOG-IgG were included. AQP4-IgG myelitis (n=46) and multiple sclerosis (MS) myelitis (n=26) patients were used as a comparative group. Outcome variables included modified Rankin score (mRS) and need for gait aid. MRI features were compared in a blinded fashion by a neuro-radiologist.

Results

Fifty-four MOG-IgG myelitis patients were included. Isolated transverse myelitis was the initial clinical presentation in 29 (53.5%) and 10 (19%) were initially diagnosed as viral/post-viral acute flaccid myelitis. Certain features favoring MOG-IgG over AQP4-IgG or MS myelitis were T2-signal abnormality confined to gray matter (sagittal line and axial H-sign) and lack of enhancement (p<0.05). Longitudinally-extensive T2-lesions were of similar frequency in MOG-IgG and AQP4-IgG myelitis (80% vs 79%; p=0.52) but not found in MS. More than one spinal cord lesion and conus involvement was more frequent with MOG-IgG than AQP4-IgG but did not differ from MS. Wheelchair dependence at myelitis nadir occurred in a third of MOG-IgG and AQP4-IgG patients but never with MS. However, recovery was better with MOG-IgG than AQP4-IgG patients.

Conclusions Myelitis is an early manifestation of MOG-IgG related disease and may have a clinical phenotype of acute flaccid myelitis. We identified clinical and MRI attributes that give a clue to MOG-IgG myelitis and help distinguish it from AQP4-IgG and MS myelitis.

Authors/Disclosures
Divyanshu Dubey, MD, FAAN (Mayo Clinic) PRESENTER	The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.
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 UCB. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. 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. 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 Arialys. 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. The institution of Dr. Pittock has received research support from Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from F. Hoffman/LaRoche/Genentech. 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.
Karl Krecke	Karl Krecke has nothing to disclose.
	No disclosure on file
Elia Sechi, MD (University of Sassari)	Dr. Sechi has nothing to disclose.
Nicholas L. Zalewski, MD (Mayo Clinic)	Dr. Zalewski has nothing to disclose.
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.
Eslam H. Shosha, MD (KKH-College of Medicine-Majmaah University)	Dr. Shosha has nothing to disclose.
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.
James Fryer	James Fryer has received intellectual property interests from a discovery or technology relating to health care.
Alfonso S. Lopez, MD (Mayo Clinic)	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 Horizon. 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 Genentech .
John Chen	John Chen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB. John Chen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen.
Jiraporn Jitprapaikulsan, MD (Faculty of Medicine Siriraj Hospital)	Dr. Jitprapaikulsan has nothing to disclose.
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.
Avi Gadoth, MD (Tel-Aviv Medical Center)	Dr. Gadoth has received intellectual property interests from a discovery or technology relating to health care.
Mark Keegan, MD, FAAN (Mayo Clinic)	Dr. Keegan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Keegan has received publishing royalties from a publication relating to health care. Dr. Keegan has received publishing royalties from a publication relating to health care.
Jan-Mendelt Tillema, MD (Mayo Clinic)	Dr. Tillema has nothing to disclose.
Elie Naddaf, MD (Mayo Clinic)	Dr. Naddaf has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Expert Connect. The institution of Dr. Naddaf has received research support from NIAMS. The institution of Dr. Naddaf has received research support from American Neuromuscular Foundation. The institution of Dr. Naddaf has received research support from Fulcrum therapeutics. The institution of Dr. Naddaf has received research support from Abcuro. The institution of Dr. Naddaf has received research support from Cabaletta .
Marc C. Patterson, MD, FRACP, FAAN	Dr. Patterson has received personal compensation for serving as an employee of IntraBio. The institution of Dr. Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Orphazyme /KemPharm/Zevra. Dr. Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Azafaros. Dr. Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for IntraBio. Dr. Patterson 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 Sage. Dr. Patterson has stock in IntraBio. The institution of Dr. Patterson has received research support from Glycomine. The institution of Dr. Patterson has received research support from Orphazyme /KemPharm/Zevra. The institution of Dr. Patterson has received research support from Idorsia. The institution of Dr. Patterson has received research support from Shire-Takeda. Dr. Patterson has received publishing royalties from a publication relating to health care. Dr. Patterson has received publishing royalties from a publication relating to health care.
Kevin Messacar, MD	No disclosure on file
Kenneth L. Tyler, MD, FAAN (University of Colorado School of Medicine)	Dr. Tyler has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Protagonist Therapeutics, Newark CA. Dr. Tyler has received personal compensation in the range of $50,000-$99,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Neurological Association/Wiley. Dr. Tyler has received intellectual property interests from a discovery or technology relating to health care. Dr. Tyler has received publishing royalties from a publication relating to health care. Dr. Tyler has a non-compensated relationship as a Director (ex officio) with American Neurological association that is relevant to AAN interests or activities. Dr. Tyler has a non-compensated relationship as a Director with International Society for Neurovirology that is relevant to AAN interests or activities.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic)	The institution of Dr. Flanagan 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. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. The institution of Dr. Flanagan 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. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from Viela Bio. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities.

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