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Abstract Details

Rituximab Improves Recovery Trajectories in Pediatric N-Methyl-D-Aspartate Receptor Encephalitis
Autoimmune Neurology
P2 - Poster Session 02 (3:00 PM-4:00 PM)
C14 - Autoimmune Encephalitis: Pathophysiology to Treatment (10:51 AM-11:01 AM)
1-053

Early first-line immunotherapy improves outcomes in pediatric N-methyl-D-aspartate receptor encephalitis (pNMDARE), while consensus guidelines reserve second-line therapies (e.g., rituximab) for refractory cases. 
We evaluated outcomes in pNMDARE with rituximab treatment.
Retrospective pNMDARE cohort study across 18 institutions from the CONNECT (CONquering Neuroinflammation and Epilepsies ConsorTium) registry. Modified Rankin Scale (mRS) was evaluated at presentation, 3, 6, 12, and 24 months. Exact matching (2:1 without replacement) accounted for corticosteroids, intravenous immunoglobulin, plasmapheresis, cyclophosphamide, and presenting mRS. Generalized linear mixed models were used; adjusted models incorporated time and nested random effects.
Among 219 patients, 103 were matched (65 rituximab-treated, 38 no-rituximab). Baseline characteristics and unadjusted comparisons of mRS at each timepoint were similar. Severe disability (mRS 5) at 24 months occurred only in the no-rituximab group (n=2). In adjusted analyses across follow-ups (n=335 observations), rituximab-treated patients improved more over time (β=-1.47, 95%CI [-2.71, -0.22], p=.022).  Rituximab effect varied by corticosteroid use (β=1.81, 95%CI [0.53, 3.08], p=0.006) and over time (β=-.23 per month, 95%CI [-0.45, -0.01], p=0.044). In post-hoc contrasts versus neither treatment, rituximab-alone showed the greatest mRS improvement (-2.30; 95%CI [-3.30, -1.30], p<.001), followed by rituximab + corticosteroids (-2.16; 95%CI [-3.14, -1.18], p<.001), and corticosteroids-alone (-1.66; 95%CI [-2.90, -0.43], p=.009). mRS did not differ between rituximab + corticosteroids versus rituximab-alone (0.14; 95%CI [-0.20, 0.48], p=.413) or corticosteroids-alone (-0.50; 95%CI [-1.44, 0.45], p=.297).
Rituximab treatment in pNMDARE improves recovery trajectory, although benefits may be modulated by other treatment effects. Prospective large-scale studies are needed to validate findings and examine effects of individual immunotherapies, timing of treatments, and relapse rates.
Authors/Disclosures
Alexandra B. Kornbluh, MD (Children's National Hospital)
PRESENTER 		Dr. Kornbluh has nothing to disclose.
Ilana L. Kahn, MD (Childrens National Medical Center) Dr. Kahn has nothing to disclose.
Leigh N. Sepeta, PhD (Children'S National Health System) Dr. Sepeta has received personal compensation in the range of $500,000-$999,999 for serving as a Grant PI with Nih. Dr. Sepeta has a non-compensated relationship as a Special volunteer with Nih that is relevant to AAN interests or activities.
Zhulin He (Emory University) Zhulin He has nothing to disclose.
James N. Brenton, MD, FAAN Dr. Brenton has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for I-ACT on a Novartis sponsored project. The institution of Dr. Brenton has received research support from NIH/NINDS. The institution of Dr. Brenton has received research support from Autoimmune Encephalitis Alliance. Dr. Brenton has received publishing royalties from a publication relating to health care. Dr. Brenton has received personal compensation in the range of $500-$4,999 for serving as a Grant Reviewer with Department of Defense. Dr. Brenton has received personal compensation in the range of $0-$499 for serving as a Grant Reviewer with NIH. Dr. Brenton has received personal compensation in the range of $0-$499 for serving as a Grant Reviewer with FDA.
Jennifer H. Yang, MD (Rady Childrens Hospital/UCSD) Dr. Yang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Yang has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for BMJ Case Reports. The institution of Dr. Yang has received research support from Pediatric Epilepsy Research Foundation. The institution of Dr. Yang has received research support from NIH. The institution of Dr. Yang has received research support from Rady Foundation.
Coral M. Stredny, MD (Children's Hospital Boston) Dr. Stredny has stock in Proctor and Gamble. The institution of Dr. Stredny has received research support from Pediatric Epilepsy Research Foundation.
Ryan Kammeyer, MD (Childrens Hospital Colorado) The institution of Dr. Kammeyer has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen. The institution of Dr. Kammeyer has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Dr. Kammeyer has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Ogborn-Mihm Trial Lawyers. The institution of Dr. Kammeyer has received research support from Rocky Mountain Multiple Sclerosis Center.
Kristen Fisher, DO (Baylor College of Medicine) Dr. Fisher has nothing to disclose.
Alexander Sandweiss, MD, PhD Dr. Sandweiss has nothing to disclose.
Ayush Gupta, MBBS Dr. Gupta has nothing to disclose.
Michael Sweeney, MD, FAAN (University of Louisville) Dr. Sweeney has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Genentech. Dr. Sweeney has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis.
Varun Kannan, MD (Emory/CHOA) Dr. Kannan has nothing to disclose.
Catherine E. Otten, MD The institution of Dr. Otten has received research support from CDC.
NgocHanh H. Vu, MD (Vanderbilt University, Child Neurology) Dr. Vu has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Syneos Health.
Jonathan Santoro, MD (Department of Neurology, Children's Hospital Los Angeles) Dr. Santoro has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB. Dr. Santoro has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Cycle Pharma. Dr. Santoro has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Dianthus. Dr. Santoro has received personal compensation in the range of $500-$4,999 for serving as a Consultant for National Down Syndrome Society.
Karla Robles Lopez (University of Texas at Austin/DMS) Karla Robles Lopez has nothing to disclose.
Robert C. Goodrich III, MD Dr. Goodrich has nothing to disclose.
Scott I. Otallah, MD (Wake Forest) Dr. Otallah has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Merck.
Janetta L. Arellano, MD (CHOC) Dr. Arellano has nothing to disclose.
Lydia Marcus, MD (University of Alabama Medical Center, Child Neurology) Dr. Marcus has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Ragsdale LLC. The institution of Dr. Marcus has received research support from NIAID.
Lileth Joy H. Mondok, MD (MCW - Children'S Hospital of Wisconsin) Dr. Mondok has nothing to disclose.
Andrew Christiana, MD (NYU) Dr. Christiana has nothing to disclose.
Morgan Morris (Emory University) Morgan Morris has nothing to disclose.
Mark Gorman, MD The institution of Dr. Gorman has received research support from Pfizer. The institution of Dr. Gorman has received research support from Roche / Genetech .
Yike Jiang Yike Jiang has nothing to disclose.
Melissa A. Wright, MD (University of Utah) Dr. Wright has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis .
Timothy Erickson (Texas A&M University) Timothy Erickson has received research support from Centers for Disease Control and Prevention.
Eyal Muscal Eyal Muscal has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for sobi. An immediate family member of Eyal Muscal has stock in pfizer.
Kristy Murray (Baylor College of Medicine) Kristy Murray has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Valneva. The institution of Kristy Murray has received research support from NIH. The institution of Kristy Murray has received research support from CDC. Kristy Murray has received personal compensation in the range of $10,000-$49,999 for serving as a Assoc Scientific Program Chair with American Society of Tropical Medicine and Hygiene.
Manikum Moodley, MD, FAAN Dr. Moodley has nothing to disclose.
Duriel I. Hardy, MD (Dell Children's Specialty Pavillian) Dr. Hardy has nothing to disclose.
Claude Steriade, MD (NYU) The institution of Dr. Steriade has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for The Epilepsy Study Consortium. Dr. Steriade has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Dynamed. Dr. Steriade has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for DOJ. The institution of Dr. Steriade has received research support from NIH. Dr. Steriade has received personal compensation in the range of $500-$4,999 for serving as a Consultant with Epitel. Dr. Steriade has received personal compensation in the range of $500-$4,999 for serving as a Consultant with Jazz Pharmaceuticals. Dr. Steriade has received personal compensation in the range of $10,000-$49,999 for serving as a Speakers Bureau with SK Life Sciences. Dr. Steriade has received personal compensation in the range of $5,000-$9,999 for serving as a Speakers Bureau with Neurelis. Dr. Steriade has received personal compensation in the range of $5,000-$9,999 for serving as a Advisory Board with Xenon Pharmaceuticals.
Grace Gombolay, MD, FAAN (Emory University/Children'S Healthcare of Atlanta) Dr. Gombolay has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. The institution of Dr. Gombolay has received research support from CDC. The institution of Dr. Gombolay has received research support from NIH. Dr. Gombolay has a non-compensated relationship as a Board of Trustee with National MS Society -Georgia chapter that is relevant to AAN interests or activities.