Abstract Details

Title Cerebral Attacks in MOG Antibody-Associated Disease: Clinical Spectrum and Fulfillment of ADEM criteria

Topic Autoimmune Neurology

Presentation(s) S40 - Autoimmune Neurology: Stiff Person Syndrome/GAD65 Neurological Autoimmunity, CIDP, and MOGAD (4:42 PM-4:54 PM)

Poster/Presentation
Number 007

Background The clinical spectrum of MOGAD cerebral attacks has not been fully elucidated and it is unclear what proportion adhere to ADEM criteria.

Objective To characterize cerebral attacks of myelin oligodendrocyte-glycoprotein-antibody-associated disease (MOGAD) and assess for fulfillment of acute-disseminated encephalomyelitis (ADEM) criteria.

Design/Methods We retrospectively searched our Mayo Clinic MOGAD database, and inclusion criteria were: 1) Symptomatic cerebral attack; and 2) Abnormal brain MRI; 3) MOG-IgG positivity. We assessed if included children and adults fulfilled current ADEM criteria of: A) First, polyfocal clinical central nervous system demyelinating event; B) encephalopathy (not explained by fever); C) no new clinical/MRI findings beyond 3 months; D) abnormal brain MRI (typically large, poorly-demarcated T2-hyperintensities involving white or deep gray matter, while T1-hypointense lesions are rare).

Results We included 90 patients. The median age was 23 (range, 2 -75), and 61% were female. In 22 (24%), recurrent cerebral attacks occurred. Clinical manifestations from the first cerebral attack included: headache, 51 (57%); brainstem symptoms, 41 (46%); encephalopathy, 34 (38%); cerebellar symptoms, 36 (40%), seizures, 17 (19%). In 16 patients (18%), the attack fell within the spectrum of cerebral cortical encephalitis. Concomitant myelitis was noted in 44 and 17 had concomitant optic neuritis. Acute MRI images were available for re-review in 81/90 and abnormalities included: poorly demarcated lesions, 55/81 (68%); deep gray matter lesions, 35/81 (43%); cerebral T1-hypointense lesions 46/81 (57%). In our cohort, 7 (8%) fulfilled ADEM criteria, and 83 (92%) did not due to ≥1 of: subsequent relapse, 58 (64%); absence of encephalopathy, 38 (42%); preceding demyelinating attack, 27 (30%); encephalopathy with fever, 15 (17%), and single lesion 4 (5%).

Conclusions Cerebral MOGAD attacks have a wide spectrum and usually do not fulfill ADEM criteria when retrospective applied. Future revisions of ADEM criteria might consider a disease-associated ADEM category to better capture MOGAD cerebral attacks.

Authors/Disclosures
Sara Hooshmand, MD (Mayo Clinic) PRESENTER	Dr. Hooshmand has nothing to disclose.
Xiaoyang Li, MD	Dr. Li has nothing to disclose.
John Chen	No disclosure on file
Vyanka Redenbaugh, MB BCh BAO (Mayo Clinic)	Dr. Redenbaugh has nothing to disclose.
Samantha Banks, MD (Mayo Clinic)	Dr. Banks has nothing to disclose.
Laura Cacciaguerra, MD, PhD (Mayo Clinic)	Dr. Cacciaguerra has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Cacciaguerra has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for BMS Celgene. Dr. Cacciaguerra has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi. Dr. Cacciaguerra has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for BIOMEDIA.
Cristina Valencia Sanchez, MD (Mayo Clinic Arizona)	Dr. Valencia Sanchez has nothing to disclose.
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 .
Elia Sechi, MD (University of Sassari)	Dr. Sechi has nothing to disclose.
Jan-Mendelt Tillema, MD (Mayo Clinic)	Dr. Tillema has nothing to disclose.
Sean J. Pittock, MD (Mayo Clinic Dept of Neurology)	Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech, Inc.. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sage Therapeutics, Inc.. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astellas. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Prime Therapeutics. 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 Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MedImmune/Viela Bio. 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 $500-$4,999 for serving as a Consultant for Roche/Genentech. 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, Inc. 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 Hoffman/LaRoche AG. 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 Genetech. 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 F. Hofman/LaRoche. 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 research support from Grifols. The institution of Dr. Pittock has received research support from NIH. The institution of Dr. Pittock has received research support from Viela Bio/MedImmune/Horizon. The institution of Dr. Pittock has received research support from Alexion Pharmaceuticals. The institution of Dr. Pittock has received research support from F. Hoffman/LaRoche/Genentech. The institution of Dr. Pittock has received research support from NovelMed. The institution of Dr. Pittock has received research support from AstaZeneca. 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.
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.