Abstract Details

Title Acute Symptomatic Seizures Secondary to Myelin Oligodendrocyte Glycoprotein Antibody-associated Disorder

Topic Autoimmune Neurology

Presentation(s) S3 - Autoimmune Neurology 1: Mechanisms of Disease, Clinical Practice (1:48 PM-2:00 PM)

Poster/Presentation
Number 005

Background Details of seizure presentations and long-term seizure outcome is limited in patients with MOGAD.

Objective

To report clinical presentations and outcomes of patients with seizure and myelin oligodendrocyte glycoprotein antibody-associated disorder (MOGAD).

Design/Methods

We identified 361 patients in our MOGAD cohort and retrospectively reviewed the medical records of 22 patients that met criteria for MOGAD associated seizures (6%).

Results

The majority of patients (n=18, 82%) had pediatric disease onset and the predominant seizure type was focal (n=13, 60%), of which 11 (50%) had motor manifestations. Eight patients (36%) had definite impaired awareness during their seizures, and 11 (50%) experienced bilateral tonic-clonic seizures. Three patients (13%) developed status epilepticus.

All patients met criteria for encephalitis at onset of seizures. The most frequent clinico-radiologic phenotype was cerebral cortical encephalitis (CCE) (n=14, [63%]; unilateral n=9 [63%] and bilateral n=5 [23%]) followed closely by acute disseminated encephalomyelitis (ADEM) (n=12, 54%). Five patients (23%) had both ADEM and CCE features. Three patients (13%) had coexistence of NMDA-R IgG. Histopathological analysis (n=2) revealed intracortical and subpial cortical demyelination. Only 8/22 patients (36%) continued to report seizures two weeks after the acute ADEM/CCE/meningoencephalitis attack, and 3/22 (16%) developed drug resistant epilepsy. One of these patients became seizure free following temporal lobectomy and excision of hippocampus. The majority, 17/22 (77%) had MOGAD relapses, commonly optic neuritis.

Conclusions Seizures were infrequent and typically remitted over time in this predominantly pediatric cohort presenting with CCE/ADEM meningoencephalitis MOGAD phenotype. The majority do not require maintenance/chronic anti-seizure medications. Focal motor seizures with or without impaired awareness are the most common seizure type. Seizures occurred concomitant with the onset of encephalitis. Intracortical and subpial cortical demyelination may explain the seizure predisposition in these patients compared to other demyelinating disorders. Although seizure recurrence is uncommon, most patients have clinical relapses resembling other MOGAD phenotypes.

Authors/Disclosures
Mayra Montalvo Perero, MD (University of Florida) PRESENTER	Dr. Montalvo Perero has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AMGEN.
Jamal F. Khattak, MD	Dr. Khattak has nothing to disclose.
Vyanka Redenbaugh, MB BCh BAO (Mayo Clinic)	Dr. Redenbaugh has nothing to disclose.
Yong Guo	No disclosure on file
Cristina Valencia Sanchez, MD (Mayo Clinic Arizona)	Dr. Valencia Sanchez has nothing to disclose.
Jan-Mendelt Tillema, MD (Mayo Clinic)	Dr. Tillema 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.
John Chen	No disclosure on file
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.
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.
Jeffrey W. Britton, MD, FAAN (Mayo Graduate School of Medicine)	Dr. Britton has received personal compensation in the range of $0-$499 for serving as a Online course with American Clinical Neurophysiology Society.
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.
Divyanshu Dubey, MD, FAAN (Mayo Clinic)	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.