Relapses, Comorbidities, and Predictors of Outcome in Anti‐ <scp> GABA <sub>A</sub> </scp> Receptor Encephalitis

OBJECTIVES: R) encephalitis, and assess the utility of LIM-domain-only-protein 5 (LMO5) antibodies as tumor markers. METHODS: R antibodies were confirmed by 2 techniques in serum or cerebrospinal fluid. Long-term outcomes were defined as good (modified Rankin scale, mRS = 0-1) or poor (mRS 2-5) at ≥12 months. LMO5 antibodies were assessed by cell-based assays and Western blot. RESULTS: Thirty-three patients were identified (4 children, 29 adults; median age, 5.5 and 60 years; 61% male). Ten patients (10/32, 31%) had concurrent systemic autoimmunity. Adults presented with seizures and cognitive/behavioral symptoms, often with thymoma, gastrointestinal, or other tumors (18/33, 55%), whereas children frequently had seizures and ataxia with cerebellar MRI lesions. Multifocal T2/fluid-attenuated inversion recovery hyperintensities were present at onset in 23 of 31 (74%) or developed later in those with absent or single lesions. Lesions showed dynamic changes, suggesting ongoing inflammation even without clinical correlate. Relapses occurred in 17 of 31 (55%, all adults) and were associated with older age (p = 0.02) and lack of second-line immunotherapy (p = 0.02). Four patients (4/33, 12%) died. After a 32.5-month median follow-up, 9 of 20 (45%) had persistent cognitive deficits, and 6 of 20 (30%) had a poor outcome, which was associated with relapses (p = 0.04). LMO5 antibodies were absent in patients and controls. INTERPRETATION: R encephalitis shows age-dependent presentations, most commonly seizures. MRI reveals dynamic changes consistent with an ongoing "clinically silent" inflammation. Relapses and cognitive sequelae are common and associate with not receiving second-line immunotherapy. LMO5 antibodies lack tumor-predictive value. ANN NEUROL 2026.

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Journal
Annals of Neurology
Published
2026-04-12
DOI
https://doi.org/10.1002/ana.78208
Primary Topic
Autoimmune Neurological Disorders and Treatments
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article
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article

Relapses, Comorbidities, and Predictors of Outcome in Anti‐ GABA A Receptor Encephalitis

Eugenia Martínez‐Hernández, Elianet Fonseca, Takashi Araga, Lídia Sabater et al.
Annals of Neurology
Autoimmune Neurological Disorders and Treatments
article

Relapses, Comorbidities, and Predictors of Outcome in Anti‐ GABA A Receptor Encephalitis

Eugenia Martínez‐Hernández, Elianet Fonseca, Takashi Araga, Lídia Sabater, J. D. M. de Vries, Lívia Almeida Dutra, Jesús Planagumà, Florian Lamblin, Alberto Vogrig, Masanori Kurihara, N Cunha, Takahiro Iizuka, Ana Beatriz Serafim, Marianna Spatola, Romana Höftberger, Chiara Milano, Esther Aguilar, Mateus Mistieri Simabukuro, Laura Marmolejo, Yuto Uchida, Takashi Miwa, Yuki Fukami, Kenichi Kaida, M. Guasp, Thais Armangué, Maria Lucia Schmitz Ferreira Santos, Francesc Graus, Raffaele Iorio, Claudia Papi, Anti‐GABAAR encephalitis study group, Luz Victoria Garcia, Maarten J. Titulaer, Josep Dalmau, Satoshi Okada
article en

Abstract

OBJECTIVES: R) encephalitis, and assess the utility of LIM-domain-only-protein 5 (LMO5) antibodies as tumor markers. METHODS: R antibodies were confirmed by 2 techniques in serum or cerebrospinal fluid. Long-term outcomes were defined as good (modified Rankin scale, mRS = 0-1) or poor (mRS 2-5) at ≥12 months. LMO5 antibodies were assessed by cell-based assays and Western blot. RESULTS: Thirty-three patients were identified (4 children, 29 adults; median age, 5.5 and 60 years; 61% male). Ten patients (10/32, 31%) had concurrent systemic autoimmunity. Adults presented with seizures and cognitive/behavioral symptoms, often with thymoma, gastrointestinal, or other tumors (18/33, 55%), whereas children frequently had seizures and ataxia with cerebellar MRI lesions. Multifocal T2/fluid-attenuated inversion recovery hyperintensities were present at onset in 23 of 31 (74%) or developed later in those with absent or single lesions. Lesions showed dynamic changes, suggesting ongoing inflammation even without clinical correlate. Relapses occurred in 17 of 31 (55%, all adults) and were associated with older age (p = 0.02) and lack of second-line immunotherapy (p = 0.02). Four patients (4/33, 12%) died. After a 32.5-month median follow-up, 9 of 20 (45%) had persistent cognitive deficits, and 6 of 20 (30%) had a poor outcome, which was associated with relapses (p = 0.04). LMO5 antibodies were absent in patients and controls. INTERPRETATION: R encephalitis shows age-dependent presentations, most commonly seizures. MRI reveals dynamic changes consistent with an ongoing "clinically silent" inflammation. Relapses and cognitive sequelae are common and associate with not receiving second-line immunotherapy. LMO5 antibodies lack tumor-predictive value. ANN NEUROL 2026.

Annals of Neurology
Università Cattolica del Sacro Cuore (IT), University of Pisa (IT), University of Udine (IT), Hospital Sant Joan de Déu Barcelona (ES), Tokyo Medical University (JP), St. Marianna University School of Medicine (JP), University of Reunion Island (RE), Hospital Israelita Albert Einstein (BR), Erasmus MC (NL), Agostino Gemelli University Polyclinic (IT), Centre Hospitalier Universitaire de La Réunion (RE), Instituto do Câncer do Estado de São Paulo (BR), Comprehensive Cancer Center Vienna (AT), Tokyo Dental College Ichikawa General Hospital (JP), Instituto Nacional de Salud del Niño (PE), Hospital Erasto Gaertner (BR), University of Tokyo Hospital (JP), Centro de Investigación Biomédica en Red (ES), Hospital Clínic de Barcelona (ES), Osaka Minami Medical Center (JP), Ospedale Santa Maria della Misericordia di Udine (IT), Peuplements végétaux et bioagresseurs en milieu tropical (RE), Hospital Universitário de Brasília (BR), Istituti di Ricovero e Cura a Carattere Scientifico (IT), Fundació Clínic per a la Recerca Biomèdica (ES), Consorci Institut D'Investigacions Biomediques August Pi I Sunyer (ES), Instituto de Investigación de Enfermedades Raras (ES), Nagoya University (JP), Kitasato University (JP), Medical University of Vienna (AT), University of Pennsylvania (US), Saitama Medical University (JP)
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Autoimmune Neurological Disorders and Treatments
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