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Abstract Details

Investigations on Myelin Oligodendrocyte Glycoprotein Autoimmunity in Children
Autoimmune Neurology
S16 - Autoimmune Neurological Disorders: Diagnosis, Biomarkers, and Epidemiology (1:00 PM-1:12 PM)
001
To report the spectrum of MOG-Ab-associated syndromes in children
Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) have been mainly associated with demyelinating syndromes
Prospective observational study of 116 children with MOG-Ab-associated syndromes recruited in two different cohorts in 40 centers in Spain: Cohort-A, demyelinating disorders; Cohort-B, encephalitis other than acute disseminated encephalomyelitis (ADEM). Outcome was assessed with the modified Rankin scale (mRS). We used Fisher-Exact and Wilcoxon-rank sum tests to analyze clinical features, and survival COX regression to analyze time to antibody negativity

116 patients had MOG-Ab including 94 (39%) of 239 patients from Cohort-A and 22 (7%) of 296 from Cohort-B; 57 (49%) were female, median age 6.2 years (IQR 3.7-10.0). Presenting MOG-Ab-associated syndromes included: ADEM (68, 59%); encephalitis other than ADEM (22, 19%); optic neuritis (20, 17%); myelitis (13, 11%), neuromyelitis optica spectrum disorders (6, 5%), and other (9, 8%). Among autoimmune encephalitis other than ADEM, MOG-Ab were the most frequent autoantibodies (22 [34%] of 64,) surpassing all neuronal-antibodies combined (21, 33%). After a median follow-up of 42 months (IQR 22-67), 33 (28%) of the 116 patients had relapses, including 17 (17%) of 100 patients diagnosed at first episode. First line immunotherapy was used in 100 (86%) patients at initial diagnosis, and 32 (97%) of 33 at relapses. Rituximab was predominantly used at relapses (11, 33%). 99 (85%) patients had substantial recovery (mRS<2), and 17 (14%) moderate-severe deficits (mRS>2; 1 died). Two phenotypes of poor prognosis included: ADEM-like relapses progressing to leukodystrophy-like features; and extensive cortical encephalitis evolving to atrophy. Time to antibody negativity was longer in patients with relapses (HR, 0.18; 95%CI, 0.05-0.57).

The spectrum of pediatric MOG-Ab-associated syndromes is wider than expected and includes demyelinating syndromes and encephalitis. Identification of these disorders is important because it has clinical and prognostic implications.
Authors/Disclosures
Thais Armangue, MD (IDIBAPS-HClinic)
PRESENTER 		Dr. Armangue 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. Armangue has received research support from ISCIII(Spanish institute of health) -PI21/00316, Marato TV3, La Caixa Research Foundadion, Pablove Foundation (689368), Torrons Vicens Foundation (PFNR0144), 2021 Invest AEP.
No disclosure on file
Eugenia Martinez-Hernandez, MD (Hospital Clinic Barcelona) Dr. Martinez-Hernandez 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. Martinez-Hernandez 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. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. The institution of Dr. Martinez-Hernandez has received research support from Instituto de Salud Carlos III. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings with Sanofi. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings and speaker honoraria with UCB. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a speaker honoraria with Alexion. Dr. Martinez-Hernandez has received personal compensation in the range of $0-$499 for serving as a speaker honoraria with Terumo. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings with Janssen. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings with CSL Behring.
Maria Sepúlveda No disclosure on file
No disclosure on file
Georgina Arrambide, MD, PhD (Cemcat, Vall d´Hebron University Hospital) The institution of Dr. Arrambide 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. Arrambide has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Merck. The institution of Dr. Arrambide has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Dr. Arrambide has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Sage. The institution of Dr. Arrambide has received research support from Instituto de Salud Carlos III. Dr. Arrambide has received personal compensation in the range of $500-$4,999 for serving as a Travel support for scientific meetings with Novartis. Dr. Arrambide has received personal compensation in the range of $500-$4,999 for serving as a Travel support for scientific meetings with Roche. Dr. Arrambide has received personal compensation in the range of $500-$4,999 for serving as a Travel support for scientific meetings with ECTRIMS. Dr. Arrambide has received personal compensation in the range of $500-$4,999 for serving as a Travel support for scientific meetings with EAN. Dr. Arrambide has a non-compensated relationship as a Member of the Executive Committee with iWiMS that is relevant to AAN interests or activities. Dr. Arrambide has a non-compensated relationship as a Member of the steering committee with BioMS-eu that is relevant to AAN interests or activities. Dr. Arrambide has a non-compensated relationship as a Member of the steering group with MOGAD Eugene Devic European Network (MEDEN) that is relevant to AAN interests or activities. Dr. Arrambide has a non-compensated relationship as a Member of the editorial and scientific committee with Acta Neurológica Colombiana that is relevant to AAN interests or activities.
No disclosure on file
Mar Tintoré, MD, PhD The institution of Mar Tintoré, MD, PhD has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. The institution of Mar Tintoré, MD, PhD has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Merck. The institution of Mar Tintoré, MD, PhD has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. The institution of Mar Tintoré, MD, PhD has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Roche . The institution of Mar Tintoré, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi Genzyme . The institution of Mar Tintoré, MD, PhD has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Teva. The institution of Mar Tintoré, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Viela bio. The institution of Mar Tintoré, MD, PhD has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Janssen. Mar Tintoré, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Mar Tintoré, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Mar Tintoré, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Mar Tintoré, MD, PhD 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 Mar Tintoré, MD, PhD has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janssen. The institution of Mar Tintoré, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Parexel and UCB Biopharma. Mar Tintoré, MD, PhD 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 Multiple Sclerosis Journal: Experimental, Translational and Clinical. The institution of Mar Tintoré, MD, PhD has received research support from Fundació La Marató de TV3. The institution of Mar Tintoré, MD, PhD has received research support from Instituto de Salud Carlos III. The institution of Mar Tintoré, MD, PhD has received research support from Instituto de Salud Carlos III. The institution of Mar Tintoré, MD, PhD has received research support from Instituto de Salud Carlos III.
Xavier Montalban, MD, PhD, FAAN (Vall Hebron University Hospital-Multiple Sclerosis Centre of Catalonia) The institution of Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. The institution of Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Merck/ EMD Serono. The institution of Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. The institution of Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Teva. The institution of Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Hoffmann-La Roche. The institution of Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Actelion/ Janssen Pharmaceuticals. The institution of Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for BMS/ Celgene. The institution of Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi/ Genzyme. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BIAL PD. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving as a Consultant for PeerVoice. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Samsung Biosys. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Zenas BioPharma. Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Actelion/ Janssen Pharmaceuticals. Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for BMS/ Celgene. Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck/ EMD Serono. Dr. Montalban 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. Montalban has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Hoffmann-La Roche. Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi-Genzyme. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Immunic Therapeutics. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Viatris/ Mylan. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sandoz. The institution of Dr. Montalban has received research support from Biogen. The institution of Dr. Montalban has received research support from Hoffmann-La Roche. The institution of Dr. Montalban has received research support from Sanofi/ Genzyme. The institution of Dr. Montalban has received research support from Merck/ EMD Serono. The institution of Dr. Montalban has received research support from Novartis. The institution of Dr. Montalban has received research support from Teva Pharmaceuticals. The institution of Dr. Montalban has received research support from Actelion/ Janssen Pharmaceuticals. The institution of Dr. Montalban has received research support from BMS/ Celgene.
Adeline Vanderver, MD, FAAN (Children'S Hospital of Philadelphia) An immediate family member of Dr. Vanderver has received personal compensation for serving as an employee of Maryland Physician Care. The institution of Dr. Vanderver has received research support from Takeda. The institution of Dr. Vanderver has received research support from Passage Bio. The institution of Dr. Vanderver has received research support from Homology. The institution of Dr. Vanderver has received research support from Eli Lilly. The institution of Dr. Vanderver has received research support from Myrtelle. The institution of Dr. Vanderver has received research support from SynaptixBio. The institution of Dr. Vanderver has received research support from PMD Foundation. The institution of Dr. Vanderver has received research support from Ionis. The institution of Dr. Vanderver has received research support from ISD . The institution of Dr. Vanderver has received research support from Boehringer-Ingelheim. The institution of Dr. Vanderver has received research support from Biogen. The institution of Dr. Vanderver has received research support from Sana. The institution of Dr. Vanderver has received research support from Affinia. The institution of Dr. Vanderver has received research support from BridgeBio. The institution of Dr. Vanderver has received research support from Orchard. The institution of Dr. Vanderver has received research support from Minoryx. The institution of Dr. Vanderver has received research support from Forge Biologics. The institution of Dr. Vanderver has received research support from Vigil. Dr. Vanderver has received intellectual property interests from a discovery or technology relating to health care. Dr. Vanderver has received intellectual property interests from a discovery or technology relating to health care.
Francesc R. Graus, MD (IDIBAPS) Dr. Graus has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for MedLink. Dr. Graus has received intellectual property interests from a discovery or technology relating to health care.
Albert Saiz (Hospital Clinico De Barcelona) Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. Albert Saiz 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. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janseen. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for novartis. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen.
Josep O. Dalmau, MD, PhD, FAAN Dr. Dalmau has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astellas Research Institute of America. Dr. Dalmau has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen Research & Development . Dr. Dalmau 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 好色先生. An immediate family member of Dr. Dalmau has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer Nature. The institution of Dr. Dalmau has received research support from Sage Therapeutics. The institution of Dr. Dalmau has received research support from Edmond J.Safra Foundation . The institution of Dr. Dalmau has received research support from La Caixa Foundation. The institution of Dr. Dalmau has received research support from Spanish Ministry of Health (ISCIII). The institution of Dr. Dalmau has received research support from Euroimmun, Inc. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care.