Abstract
Anti-GAD65 antibodies have been identified in both acute/subacute seizures (limbic encephalitis and extralimbic encephalitis) and chronic isolated epilepsy. The evidence of high serum titers and intrathecal synthesis play a fundamental role in diagnosis but poorly correlate with disease severity or response to therapies. It remains controversial whether anti-GAD65 Abs are the pathogenic entity or only serve as a surrogate marker for autoimmune disorders mediated by cytotoxic T cells. Unlike other immune-mediated epilepsy, although multiple combinations of therapeutics are used, the efficacy and prognosis of patients with GAD65-epilepsy patients are poor. Besides, GAD65-epilepsy is more prone to relapse and potentially evolve into a more widespread CNS inflammatory disorder. This article reviews the recent advances of GAD65-epilepsy, focusing on the diagnosis, epidemiology, pathophysiology, clinical features, and treatment, to better promote the recognition and provide proper therapy for this condition.
Copyright © 2020 Elsevier B.V. All rights reserved.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Antibody Specificity
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Autoantibodies / immunology*
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Autoantigens / chemistry
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Autoantigens / immunology*
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Autoimmune Diseases of the Nervous System / immunology*
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Autoimmune Diseases of the Nervous System / therapy
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Combined Modality Therapy
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Disease Progression
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Encephalitis / epidemiology
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Encephalitis / immunology*
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Encephalitis / therapy
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Epilepsy / diagnostic imaging
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Epilepsy / epidemiology
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Epilepsy / immunology*
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Epilepsy / therapy
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Epitopes / chemistry
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Epitopes / immunology
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Glutamate Decarboxylase / chemistry
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Glutamate Decarboxylase / immunology*
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Humans
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Immunoglobulins, Intravenous / therapeutic use
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Immunosuppressive Agents / therapeutic use
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Immunotherapy
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Limbic Encephalitis / immunology
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Limbic Encephalitis / therapy
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Models, Molecular
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Neuroimaging
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Paraneoplastic Syndromes, Nervous System / immunology
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Plasmapheresis
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Protein Conformation
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Psychosurgery
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T-Lymphocytes, Cytotoxic / immunology
Substances
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Autoantibodies
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Autoantigens
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Epitopes
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Immunoglobulins, Intravenous
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Immunosuppressive Agents
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Glutamate Decarboxylase
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glutamate decarboxylase 2