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Abstract Details

Clinical Features, Risk of Relapse, and Outcome in Anti-GABAA Receptor Encephalitis
Autoimmune Neurology
P9 - Poster Session 9 (11:45 AM-12:45 PM)
8-002
This study aims to report the clinical course and outcome of patients with autoimmune encephalitis with antibodies against γ-aminobutyric acid type A receptor (GABAAR).
GABAAR encephalitis is associated with prominent seizures and multifocal brain lesions. The risk of clinical relapse and long-term outcome are unknown.
We retrospectively assessed clinical information of patients with GABAAR antibodies (identified by rat brain immunohistochemistry and cell-based assay) referred to our center from worldwide between January 2016 and April 2024. Relapses were defined as onset/worsening of neurological symptoms (isolated seizures only if accompanied by new MRI changes) after ≥ 2 months of improvement/stabilization. Long-term outcome was determined in patients with a follow-up ≥ 18 months from onset, and ≥ 12 months from last clinical episode.

Twenty-nine patients were identified, 4 (14%) children and 25 (86%) adults. At first clinical episode, 28/29 (97%) patients developed seizures, evolving into status epilepticus in 16/28 (57%). In addition to seizures, 24/28 (86%) patients had other neurological symptoms, predominantly cognitive impairment (18/28, 64%). Tumors occurred in 17/29 (59%) patients (thymoma in 13/17, 76%). Brain MRI showed multifocal cortico-subcortical T2/FLAIR abnormalities in 22/27 (81%) patients; isolated cerebellar lesions were found in 3/27 (11%). Immunotherapy was administered in 25/29 (86%) patients. Sixteen of 29 (55%) patients had one or more clinical relapses (median 1, range 1-3) manifesting with seizures in 14/20 episodes (70%). Older age at onset (p=0.02) and no treatment with second-line immunotherapy (p=0.026) were associated with relapses. Four of 29 (14%) patients died. Long-term follow-up was assessable in 15 alive patients: 7 (47%) completely recovered and 8 (53%) showed partial recovery. Cognitive disturbances were the most frequent persistent neurological deficits (6/15, 40%).

In GABAAR encephalitis, relapses are not rare, often manifesting with seizure recurrence. Half of the patients with long-term follow-up were left with residual neurological deficits, mainly cognitive disturbances.
Authors/Disclosures
Claudia Papi, MD (FRCB-IDIBAPS)
PRESENTER 		Dr. Papi has nothing to disclose.
Chiara Milano, MD Dr. Milano has nothing to disclose.
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.
Mateus M. Simabukuro, MD (Hospital Das Clinicas, Sao Paulo U Scho of Med) Dr. Simabukuro has nothing to disclose.
Laura Marmolejo Alcaide Miss Marmolejo Alcaide has nothing to disclose.
Esther Aguilar, Bachelor of science Mrs. Aguilar has nothing to disclose.
Mar Guasp, MD (Hospital Clínic Barcelona - IDIBAPS) Dr. Guasp has nothing to disclose.
Raffaele Iorio, MD (Catholic University) Dr. Iorio 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. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Iorio 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. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Dianthus Therapeutics. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson & Johnson. Dr. Iorio 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. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Argenx. Dr. Iorio has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Johnson&Johnson.
Yuki Fukami, MD, PhD Dr. Fukami has nothing to disclose.
Mara Lucia Schmitz Santos, MD Prof. Santos has nothing to disclose.
Takashi Miwa, MD Dr. Miwa has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for kyowa kirin co. ltd. Dr. Miwa has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Eisai Co., Ltd..
TAKASHI ARAGA, MD, PhD Dr. ARAGA has nothing to disclose.
Yuto Uchida, MD, PhD Dr. Uchida has nothing to disclose.
Masanori Kurihara, MD, PhD Dr. Kurihara has received personal compensation in the range of $0-$499 for serving as a Consultant for SANWA KAGAKU KENKYUSHO CO.,LTD.. Dr. Kurihara has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Eisai Co., Ltd.. Dr. Kurihara has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Takeda Pharmaceutical Co., Ltd.. Dr. Kurihara has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for AbbVie Inc.. Dr. Kurihara has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Kyowa Kirin Co.,Ltd.. Dr. Kurihara has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for John Wiley & Sons, Inc.. Dr. Kurihara has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Eli Lilly and Co.. Dr. Kurihara has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Sysmex Corp.. The institution of Dr. Kurihara has received research support from Japan Society for the Promotion of Science. The institution of Dr. Kurihara has received research support from Tokyo Metropolitan Institute for Geriatrics and Gerontology. The institution of Dr. Kurihara has received research support from Japan Agency for Medical Research and Development. Dr. Kurihara has received intellectual property interests from a discovery or technology relating to health care. Dr. Kurihara has a non-compensated relationship as a sub-investigator with Biogen Inc. that is relevant to AAN interests or activities. Dr. Kurihara has a non-compensated relationship as a sub-investigator with F. Hoffmann-La Roche, Ltd. that is relevant to AAN interests or activities. Dr. Kurihara has a non-compensated relationship as a sub-investigator with CHUGAI PHARMACEUTICAL CO.,LTD. that is relevant to AAN interests or activities. Dr. Kurihara has a non-compensated relationship as a sub-investigator with Janssen Pharmaceutical K.K. that is relevant to AAN interests or activities. Dr. Kurihara has a non-compensated relationship as a sub-investigator with Eli Lilly and Company that is relevant to AAN interests or activities. Dr. Kurihara has a non-compensated relationship as a sub-investigator with Sound Wave Innovation that is relevant to AAN interests or activities. Dr. Kurihara has a non-compensated relationship as a co-investigator with Sysmex Corporation that is relevant to AAN interests or activities. Dr. Kurihara has a non-compensated relationship as a sub-investigator with Eisai Co., Ltd. that is relevant to AAN interests or activities.
Satoshi Okada, MD Dr. Okada has nothing to disclose.
Luz V. Garcia Ruiz, MD Miss Garcia Ruiz has nothing to disclose.
Ken-ichi Kaida, MD, PhD Mr. Kaida has nothing to disclose.
Natalia S. Cunha, MD (SHS Qd 2) Dr. Cunha has nothing to disclose.
Alberto Vogrig, MD, PhD (University of Udine) Dr. Vogrig has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Eisai. Dr. Vogrig has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Angelini. The institution of Dr. Vogrig has received research support from Ministero della Salute, Bando Ricerca Finalizzata.
Florian LAMBLIN (CHU DE LA RÉUNION) Mr. LAMBLIN has nothing to disclose.
Livia A. Dutra, MD (Hospital Israelita Albert Einstein) Dr. Dutra has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. The institution of Dr. Dutra has received research support from Hospital Israelita Albert Einstein. The institution of Dr. Dutra has received research support from Laboratório Fleury. Dr. Dutra has received personal compensation in the range of $0-$499 for serving as a Speaker with Roche.
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.
Juna De Vries Juna De Vries has nothing to disclose.
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.
Lidia Sabater, PhD The institution of Dr. Sabater has received research support from Instituto de Salud Carlos III.
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.
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.
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.
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.
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.