Related Experiment Video
Updated: Apr 14, 2026
Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Relapses, Comorbidities, and Predictors of Outcome in Anti-GABAA Receptor Encephalitis
Claudia Papi1,2, Chiara Milano1,3, Laura Marmolejo1
1Neuroimmunology Program, Fundació de Recerca Clínic Barcelona-Institut d'Investigacions Biomédiques August Pi i Sunyer (FRCB-IDIBAPS), University of Barcelona and Caixa Research Institute (CRI), Barcelona, Spain.
Objectives:
To characterize the magnetic resonance imaging (MRI) lesion dynamics, comorbidities, predictors of relapse, and outcomes in anti-γ-aminobutyric acid type A receptor (GABAAR) encephalitis, and assess the utility of LIM-domain-only-protein 5 (LMO5) antibodies as tumor markers.
Methods:
GABAAR 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:
Anti-GABAAR 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;100:139-150.
Insights
Anti-gamma-aminobutyric acid type A receptor (GABAAR) encephalitis presents differently in children and adults, with MRI showing dynamic inflammation. Relapses and cognitive deficits are common, especially without immunotherapy, and LIM-domain-only-protein 5 (LMO5) antibodies are not useful tumor markers.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Anti-gamma-aminobutyric acid type A receptor (GABAAR) encephalitis is an autoimmune disorder with diverse clinical manifestations.
- Understanding the disease's natural history, including MRI findings, comorbidities, and outcomes, is crucial for patient management.
Purpose of the Study:
- To characterize magnetic resonance imaging (MRI) lesion dynamics, comorbidities, relapse predictors, and outcomes in anti-GABAAR encephalitis.
- To assess the utility of LIM-domain-only-protein 5 (LMO5) antibodies as tumor markers in this condition.
Main Methods:
- Confirmed GABAAR antibodies in serum or cerebrospinal fluid using two techniques.
- Defined long-term outcomes (modified Rankin Scale, mRS) at ≥12 months.
- Assessed LMO5 antibodies via cell-based assays and Western blot.
Main Results:
- Thirty-three patients (4 children, 29 adults) were studied. Adults often presented with seizures and cognitive symptoms, frequently associated with tumors (55%). Children typically had seizures and ataxia with cerebellar MRI lesions.
- MRI showed multifocal lesions in 74% of patients, with dynamic changes suggesting ongoing subclinical inflammation. Relapses occurred in 55% of adults and were linked to older age and lack of second-line immunotherapy.
- Poor outcomes (mRS 2-5) and persistent cognitive deficits were observed in 30% and 45% of patients, respectively, associated with relapses. LMO5 antibodies were absent in all patients and controls.
Conclusions:
- Anti-GABAAR encephalitis exhibits age-dependent presentations, predominantly seizures.
- MRI findings indicate persistent, clinically silent inflammation. Relapses and cognitive sequelae are common, particularly when second-line immunotherapy is not administered.
- LMO5 antibodies do not possess tumor-predictive value in anti-GABAAR encephalitis.
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