Abstract Details

Title Cerebral cortical encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease

Topic Autoimmune Neurology

Presentation(s) P16 - Poster Session 16 (8:00 AM-9:00 AM)

Poster/Presentation
Number 9-002

Objective

To report the clinical and radiological features, and outcomes of cerebral cortical encephalitis (CCE) in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).

Background

CCE is a recently described MOGAD phenotype, characterized by cerebral cortical T2 fluid-attenuated-inversion-recovery (T2-FLAIR) hyperintense lesions on MRI.

Design/Methods

We reviewed MRIs in our MOGAD cohort and retrospectively identified patients meeting inclusion criteria of: 1) Uni- or bilateral cortical T2-FLAIR hyperintensity; 2) MOG-IgG+ by live cell-based assay; and 3) Clinical presentation compatible with MOGAD

Results

We included 17 patients (35% adults). Median age at CCE was 14 years (range, 2-45). Ten (59%) were female. CCE manifestations included: headache, 14 (82%), seizure, 12 (71%), encephalopathy, 10 (59%); fever, 9 (53%); other cortical symptoms, 9 (53%). One patient was asymptomatic (surveillance MRI). One or more clinical syndromes co-occurred (within 1 month of CCE) in 10 (59%): optic neuritis, 7; myelitis/myeloradiculitis, 2; rhombencephalitis, 2. CCE was the first attack in 12 (71%), while 5 (29%) had ≥1 prior attacks (optic neuritis, 5; myelitis, 1; acute disseminated encephalomyelitis, 1). MOG-IgG median IgG-binding-index (n=10) was 17.8 (range 3.9-163.6[reference range, <2.5]) and median end-titer (n=7) was 1:100 (range, 1:40-1:1000 [reference range, <1:20]). MRI cortical T2-hyperintensity (unilateral, 12[71%]; bilateral, 5[29%]) had adjacent leptomeningeal enhancement in 16 (94%). Eight (47%) had concomitant cerebral parenchymal T2-hyperintensities. CSF revealed: pleocytosis, 15/16 (94% [median, 44.5 WBC/µL; range, 13-505]); and negative oligoclonal bands, 14/14. Two (12%) had coexisting CSF NMDA receptor antibodies. Acute treatments of CCE included: high-dose steroids, 16 (94%) and antiseizure medications, 11 (65%). By last follow-up (median 26 months [range, 1-244]) all improved to an EDSS of ≤2 and 7 (41%) remained monophasic.

Conclusions

CCE is most common in children, usually unilateral and may occur in isolation or concomitantly with other CNS involvement. Headaches and seizures are common manifestations and leptomeningeal enhancement often coexists. CCE is steroid-responsive with favorable outcomes.

Authors/Disclosures
Cristina Valencia Sanchez, MD (Mayo Clinic Arizona) PRESENTER	Dr. Valencia Sanchez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG therapeutics. Dr. Valencia Sanchez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Dr. Valencia Sanchez has a non-compensated relationship as a member of the medical advisory board with The MOG Project that is relevant to AAN interests or activities.
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. John Chen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB.
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.
Mayra Montalvo Perero, MD (University of Florida)	Dr. Montalvo Perero has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG THERAPEUTICS. 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.
Paul M. Elsbernd, MD (Brooke Army Medical Center)	Dr. Elsbernd 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 .
Adrian Budhram, MD (London Health Sciences Centre)	Dr. Budhram has nothing to disclose.
Elia Sechi, MD (University of Sassari)	Dr. Sechi has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Sechi has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Argenx.
Amy Kunchok, MBBS (Cleveland Clinic - Mellen Centre)	Dr. Kunchok 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 Neurology:Open Access Journal .
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.
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.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic)	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 UCB. The institution of Dr. Flanagan has received research support from Roche. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Merck. 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 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. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology that is relevant to AAN interests or activities.

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