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Abstract Details

Super-Refractory Anti-NMDAR Encephalitis: Assessment of 43 Patients with Extremely Prolonged States of Minimal Consciousness
Autoimmune Neurology
P9 - Poster Session 9 (11:45 AM-12:45 PM)
8-004

To investigate patients with prolonged (≥9 months) super-refractory anti-NMDAR encephalitis (NMDARe).
In NMDARe patients who fail first-line (steroids, IVIg, plasma exchange) and second-line (rituximab and cyclophosphamide) immunotherapies, the role of third-line therapies is unclear due to late effects of previous treatments and reporting bias. 
International retrospective observational study of NMDARe patients who remained in a minimally conscious state for ≥9 months. Data were obtained by chart review and structured questionnaires.

We identified 43 NMDARe patients, median age 23 (IQR 20-32) years; 38 (88%) women. All were admitted to ICU (median stay 298 [247-379] days) due to motor agitation (37%), dyskinesias (44%), seizures (67%), hypoventilation (79%) or dysautonomia (90%). All had abnormal EEG, 28/43 (65%) brain MRI abnormalities, 37/38 (97%) pleocytosis (median 65 [27-150] WBC/µL), and 22/43 (51%) ovarian teratomas (4 bilateral). Common ICU complications included sepsis and thrombotic events; 6 patients were resuscitated from cardiac arrest. Treatment included first-line immunotherapy (42/43, 98%), second-line immunotherapy (39/43, 91%), and third-line immunotherapy (19/43, 44%) such as bortezomib (17), methotrexate (8); tocilizumab (4); daratumumab (2), and other (3). Yet, all patients remained in a state of minimal consciousness/unresponsiveness for 360 (299-780) days. After a median follow-up of 3.4 (2-6) years, 9 (21%) died, 5 (12%) remained unresponsive (mRS 5), 5 (12%) moderately-severely impaired (mRS 3-4), and 24 (56%) improved (mRS 0-2; 20 returning to premorbid activities). Among all patients who improved (n=24), only 8 received third line immunotherapy. Conversely, among all patients who did not improve (n=19), 12 received third line immunotherapies. Three patients had complete recovery after being in coma 2-4 years and without immunotherapy 13-33 months.

NMDARe can associate with extremely prolonged states of minimal consciousness, but with potential for recovery. This study shows the difficulty in assessing third line therapies in a disease as complex as NMDARe.
Authors/Disclosures
Mar Guasp, MD (Hospital Clínic Barcelona - IDIBAPS)
PRESENTER 		Dr. Guasp has nothing to disclose.
Juliette Brenner, MD (Erasmus University Medical Center) Ms. Brenner has nothing to disclose.
pauline dumez, MD Dr. dumez has nothing to disclose.
Patrice LaLive, MD Dr. LaLive has nothing to disclose.
Katharina Wurdack, MD Ms. Wurdack has nothing to disclose.
Stefan Macher Stefan Macher has nothing to disclose.
Kang Wang, MD Dr. Wang has nothing to disclose.
Gemma Olivé, MD Dr. Olivé has nothing to disclose.
Jonas H. Yeung, MD, MB, ChB, FRCP (Alice Ho Miu Ling Nethersole Hospital) Dr. Yeung has nothing to disclose.
Giovanni Di Liberto, MD, PhD Dr. Di Liberto has nothing to disclose.
Klaus-Peter Wandinger, MD (Charite, Neurology Dept) No disclosure on file
Dominik Zieglgaensberger, MD Dr. Zieglgaensberger has nothing to disclose.
Mamoru Shibata Mamoru Shibata has received personal compensation in the range of $0-$499 for serving as an officer or member of the Board of Directors for Japanese Headache Society. Mamoru Shibata has received personal compensation in the range of $0-$499 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Japanese Headache Society. Mamoru Shibata has received personal compensation in the range of $0-$499 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Japanese Society of Neurology. The institution of Mamoru Shibata has received research support from Otsuka Pharmaceutical Co. Ltd..
Eri Fukao, MD Dr. FUKAO has nothing to disclose.
Takayasu Mishima, MD Prof. Mishima has nothing to disclose.
Hiroto Nakagawa, PhD Dr. Nakagawa has nothing to disclose.
Tsubasa Takizawa, MD, PhD Prof. Takizawa has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pfizer. Prof. Takizawa has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Otsuka. Prof. Takizawa has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Eli Lilly. Prof. Takizawa has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Teijin. Prof. Takizawa has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AbbVie. Prof. Takizawa has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Daiichi Sankyo. Prof. Takizawa has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Otsuka. Prof. Takizawa has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Eli Lilly. Prof. Takizawa has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Daiichi Sankyo. Prof. Takizawa has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Amgen. Prof. Takizawa has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Sawai. Prof. Takizawa has received research support from Eli Lilly. Prof. Takizawa has received research support from JSPS KAKENHI. Prof. Takizawa has received research support from Pfizer.
Sho Shimohama The institution of Dr. Shimohama has received research support from Grant-in-Aid for JSPS Fellows.
Yoshikane Izawa, MD, PhD The institution of Dr. Izawa has received research support from Ministry of 好色先生, Culture, Sports, Science and Technology (JAPAN).
Eric Lancaster, MD, PhD (The University of Pennsylvania, Dept. of Neurology) Dr. Lancaster has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for merck. Dr. Lancaster has received intellectual property interests from a discovery or technology relating to health care. Dr. Lancaster has received personal compensation in the range of $50,000-$99,999 for serving as a Expert and Witness with US Vaccine Injury Compensation Program.
Kurt-Wolfram Suhs Kurt-Wolfram Suhs has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Kurt-Wolfram Suhs has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Kurt-Wolfram Suhs has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Kurt-Wolfram Suhs has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. The institution of Kurt-Wolfram Suhs has received research support from Bristol Myers Squibb.
Jose Maria Cabrera Maqueda Mr. Cabrera Maqueda has nothing to disclose.
Elianet Fonseca, MD (HOSPITAL CLINICO DE BARCELONA) Dr. Fonseca has nothing to disclose.
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. The institution of Dr. Martinez-Hernandez has received research support from Instituto de Salud Carlos III. The institution of Dr. Martinez-Hernandez has received research support from Fundacio La Caixa. 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 and attending meetings with Alexion. 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.
Marianna Spatola, MD, PhD, FAAN (FUNDACIÓ DE RECERCA BIOMEDICA CLÍNIC IDIBAPS) The institution of Dr. Spatola has received research support from Spanish National Health Institute (Carlos III), FIS grant. The institution of Dr. Spatola has received research support from Spanish National Institute of Health - Miguel Servet Grant. The institution of Dr. Spatola has received research support from La Caixa Foundation. The institution of Dr. Spatola has received research support from European Research Coucil.
Renaud A. Du Pasquier, MD, FAAN (Service of neurology, CHUV) Dr. Du Pasquier has nothing to disclose.
Frank Leypoldt, MD (University Hospital Schleswig-Holstein, Campus Kiel Department of Neurology) Frank Leypoldt, MD has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Roche. Frank Leypoldt, MD has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx. Frank Leypoldt, MD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. The institution of Frank Leypoldt, MD has received research support from German Ministry of Research BMBF.
Romana Hoeftberger (Medical University of Vienna) Romana Hoeftberger has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology: Neuroimmunology and Neuroinflammation. Romana Hoeftberger has received personal compensation in the range of $500-$4,999 for serving as a speaker with BMS and UCB Biopharma.
Carsten Finke, MD (Charité Berlin) Carsten Finke, MD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myer Squibb. The institution of Carsten Finke, MD has received research support from Euroimmun.
Thais Armangue, MD (IDIBAPS-HClinic) 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.
Maarten J. Titulaer, MD, PhD (Erasmus Medical Center) The institution of Dr. Titulaer has received research support from Dutch Epilepsy Foundations (NEF 19-08). The institution of Dr. Titulaer has received research support from CSL Behring. The institution of Dr. Titulaer has received research support from UCB. The institution of Dr. Titulaer has received research support from Netherlands Organisation for Scientific Research (ZonMW, Memorabel initiative and E-RARE UltraAIE) . The institution of Dr. Titulaer has received research support from Horizon Therapeutics / Amgen. The institution of Dr. Titulaer has received research support from Dioraphte (charity). The institution of Dr. Titulaer has received research support from Guidepoint Global LLC. The institution of Dr. Titulaer has received research support from ArgenX. Dr. Titulaer has received intellectual property interests from a discovery or technology relating to health care. Dr. Titulaer has received publishing royalties from a publication relating to health care.
Mateus M. Simabukuro, MD (Hospital Das Clinicas, Sao Paulo U Scho of Med) Dr. Simabukuro has nothing to disclose.
Jerome Honnorat, MD, PhD (Hospices Civils de Lyon) Jerome Honnorat, 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 LFB. The institution of Jerome Honnorat, MD, PhD has received personal compensation in the range of $0-$499 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for journal of neurology.
Takahiro Iizuka, MD (Department of Neurology, Kitasato University School of Medicine) The institution of Dr. Iizuka has received research support from EUROIMMUN Japan Co., Ltd.
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.