Abstract Details

Title Practice Variation in Treatment of NMDAR- IgG Encephalitis Across International Centers from the Clinical Outcomes in Autoimmune Encephalitis Study (COAST)

Topic Autoimmune Neurology

Presentation(s) P3 - Poster Session 3 (11:30 AM-12:30 PM)

Poster/Presentation
Number 1-021

Objective To 1) examine the baseline characteristics of patients with NMDAR-IgG encephalitis (NMDAR-IgG AE) and 2) understand practice variation in the treatment of NMDAR-IgG AE across international centers contributing to the Clinical Outcomes in Autoimmune Encephalitis Study (COAST).

Background NMDAR-IgG AE is treated with acute immunosuppressive therapy (IST). Second-line therapies like rituximab and cyclophosphamide are used in refractory cases. However, the optimal treatment regimen and duration of treatment is not established.

Design/Methods Descriptive statistics summarized baseline cohort characteristics. Outcome measures were modified rankin scale (mRS) and clinical assessment scale for autoimmune encephalitis (CASE). Computations were performed in R, version 4.1.1.

Results

In this international, multicenter study we included 126 NMDAR-IgG AE patients. The median age at onset was 27 (21, 33) and 98 (77.8 %) were female. Presenting symptoms included psychiatric dysfunction (108 (85.7%)), seizures (81 (64.3%)), and movement disorders (67 (53.2%)). Median baseline mRS was 4 (3,5), and median baseline total CASE score was 9 (6,16). Teratoma was identified in 39/112 (34.8%) patients. CSF showed leukocytosis in 77/118 (65.2%) patients. Median length of stay in the hospital was 39 (17,61) days, and 69/116 (59.5%) patients had an ICU admission for a median of 25 (12,53) days. Median time to first-line IST (IVMP, IVIG, PLEX) was 3 (2,7) weeks after symptom onset. Patients received varied second-line therapies including rituximab (76 (60.3%)), corticosteroids (30 (23.8%)), IV/oral cyclophosphamide (17 (13.5%)), mycophenolate mofetil (8, (6.3%)), azathioprine (6, (4.8%)), tocilizumab (5, (4.0%)), bortezomib (5, (4.0%)), methotrexate (1, (0.8%)) and IVIG (1 (0.8%). At the last follow up visit, patients had a median mRS of 1 (0,2) and median total CASE score of 1 (0,3).

Conclusions Multicenter international data demonstrates substantial practice variation across centers, especially regarding the type and duration of second-line therapies. Further studies are underway to determine optimal treatment regimens.

Authors/Disclosures
Nivethitha Manohar, MD (Cleveland Clinic Foundation) PRESENTER	Dr. Manohar has nothing to disclose.
Tatchaporn Ongphichetmetha	Miss Ongphichetmetha has nothing to disclose.
Meredith Dever, MPH	Ms. Dever has nothing to disclose.
Nicholas Thompson	The institution of Nicholas Thompson has received research support from EMD Serono.
Justin Abbatemarco, MD	Dr. Abbatemarco 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. Abbatemarco 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, Inc.. Dr. Abbatemarco has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Abbatemarco 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 .
Hesham A. Abboud, MD (University Hospitals Cleveland Medical Center)	Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. The institution of Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech . Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alpine Pharma. Dr. Abboud 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. Abboud 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. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cycle Pharma. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Axonics. Dr. Abboud 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. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Genentech. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for BMS. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for TG Therapeutics. The institution of Dr. Abboud has received research support from Genentech . The institution of Dr. Abboud has received research support from Novartis. The institution of Dr. Abboud has received research support from BMS. The institution of Dr. Abboud has received research support from Sanofi-Genzyme. The institution of Dr. Abboud has received research support from The Guthy-Jackson Charitable Foundation. The institution of Dr. Abboud has received research support from UCB. Dr. Abboud has received publishing royalties from a publication relating to health care.
Adrian Budhram, MD (London Health Sciences Centre)	Dr. Budhram has nothing to disclose.
Stacey Clardy, MD, PhD, FAAN (University of Utah)	Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. Dr. Clardy 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/AAN Publications. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. The institution of Dr. Clardy has received research support from Kyverna. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa, Miami, Stanford, Barrow, Advent Health Florida, Beaumont Health, CCF, Emory, Penn State Health, Mayo Clinic, Walter Reed.
Rajesh K. Gupta, MBBS (UTHealth)	Dr. Gupta has nothing to disclose.
Tirisham Gyang, MD (Ohio State Univeristy, Wexner Medical Center, Department of Neurology)	Dr. Gyang has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for EMD Serono.
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 intellectual property interests from a discovery or technology relating to health care. Dr. Flanagan has received intellectual property interests from a discovery or technology relating to health care. 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.
Rodrigo Hasbun	Rodrigo Hasbun has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biomeriaux. The institution of Rodrigo Hasbun has received research support from Biomeriaux.
Julien Hebert, MD (Toronto Western Hospital (University Health Network))	Dr. Hebert has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Holberg EEG. Dr. Hebert has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Paladin Labs. Dr. Hebert has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Gowling WLG. The institution of Dr. Hebert has received research support from Praxis Precision Medicine.
Jyh Yung Hor, MD (Penang General Hospital)	Dr. Hor has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Novartis.
Sarosh R. Irani, MD, PhD, FRCP, FEAN (Mayo Clinic)	Dr. Irani has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Alexion, Argenex, Atheneum, AZ, Clarivate, IQVIA, BioHaven therapeutics. Dr. Irani has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion US, Amgen, Argenx . Dr. Irani 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 Brain. The institution of Dr. Irani has received research support from Amgen. Dr. Irani has received intellectual property interests from a discovery or technology relating to health care. Dr. Irani has received intellectual property interests from a discovery or technology relating to health care.
Jiraporn Jitprapaikulsan, MD (Faculty of Medicine Siriraj Hospital)	Dr. Jitprapaikulsan has nothing to disclose.
Joseph C. LaPorta (University of Cincinnati - Neurology)	Mr. LaPorta has nothing to disclose.
Soon-Tae Lee, MD, PhD (Department of Neurology, Seoul National University Hospital)	Prof. Lee has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Advanced Neural Technologies. Prof. Lee has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Piehealthcare. Prof. Lee has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Salted. Prof. Lee has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche/Genentech. Prof. Lee has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx. Prof. Lee 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 Prof. Lee has received research support from Roche. Prof. Lee has received intellectual property interests from a discovery or technology relating to health care.
Ahmad Mahadeen, MD (University of Mississippi Medical Center)	Dr. Mahadeen has nothing to disclose.
Giovanna Manzano, MD (University of California Irvine)	Dr. Manzano has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gilead Sciences. Dr. Manzano has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for InfuCare Rx.
Mariano Marrodan, MD (Institute for Neurological Research Raul Carrea. Fleni)	Dr. Marrodan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Merck. Dr. Marrodan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Roche. Dr. Marrodan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for AstraZeneca. Dr. Marrodan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Novartis.
Jennifer McCombe, MD (University of Alberta)	Dr. McCombe has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Novartis. Dr. McCombe has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen.
Alexandra Muccilli, MD (Saint Michael's Hospital - Multiple Sclerosis Clinic)	Dr. Muccilli has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Muccilli 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. Muccilli has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Muccilli 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.
Amanda L. Piquet, MD, FAAN (University of Colorado)	The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genentech/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kyverna . The institution of Dr. Piquet 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/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Sands Anderson PC. Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Joe Jones Law Firm. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Cortez & Associates. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Falk Waas. The institution of Dr. Piquet has received research support from Rocky Mountain MS Center. The institution of Dr. Piquet has received research support from Roche/Genentech. The institution of Dr. Piquet has received research support from NYU. The institution of Dr. Piquet has received research support from Anokion. The institution of Dr. Piquet has received research support from UCB . The institution of Dr. Piquet has received research support from Foundation for Sarcoidosis. The institution of Dr. Piquet has received research support from Kyverna . Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Litigative Consultant with US-Dept HHS/DICP. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Autoimmune Encephalitis Alliance (AEA) that is relevant to AAN interests or activities. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Stiff Person Syndrome Research Foundation (SPSRF) 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.
Kristine H. O'Phelan, MD (University of Miami)	Dr. O'Phelan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Bard Medical. Dr. O'Phelan has a non-compensated relationship as a DSMB member SIREN network with NIH/NINDS that is relevant to AAN interests or activities.
John Probasco, MD, FAAN (The Johns Hopkins Hospital)	Dr. Probasco 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 NEJM Clinician. The institution of Dr. Probasco has received research support from Roche/Genentech.
Amy M. Quek, MBBS (National University Hospital)	The institution of Dr. Quek has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. The institution of Dr. Quek 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. Quek has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Astra Zeneca. The institution of Dr. Quek has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. The institution of Dr. Quek has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Merck. The institution of Dr. Quek has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Astra Zeneca.
Shailee S. Shah, MD	Dr. Shah 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. Dr. Shah 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. Shah 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. Shah 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. Shah has received publishing royalties from a publication relating to health care.
Andrew Solomon, MD, FAAN	Dr. Solomon 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. Solomon 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/Roche . Dr. Solomon has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kiniksa Pharmaceuticals . Dr. Solomon has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squibb. Dr. Solomon has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. The institution of Dr. Solomon has received research support from Bristol Meyers Squibb.
Arun Venkatesan, MD, PhD (Johns Hopkins Hospital)	The institution of Dr. Venkatesan has received research support from NIH. The institution of Dr. Venkatesan has received research support from U.S. DOD.
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 .

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