Immune-mediated epilepsy with GAD65 antibodies

J Neuroimmunol. 2020 Apr 15:341:577189. doi: 10.1016/j.jneuroim.2020.577189. Epub 2020 Feb 14.

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antibody Specificity
  • Autoantibodies / immunology*
  • Autoantigens / chemistry
  • Autoantigens / immunology*
  • Autoimmune Diseases of the Nervous System / immunology*
  • Autoimmune Diseases of the Nervous System / therapy
  • Combined Modality Therapy
  • Disease Progression
  • Encephalitis / epidemiology
  • Encephalitis / immunology*
  • Encephalitis / therapy
  • Epilepsy / diagnostic imaging
  • Epilepsy / epidemiology
  • Epilepsy / immunology*
  • Epilepsy / therapy
  • Epitopes / chemistry
  • Epitopes / immunology
  • Glutamate Decarboxylase / chemistry
  • Glutamate Decarboxylase / immunology*
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Immunotherapy
  • Limbic Encephalitis / immunology
  • Limbic Encephalitis / therapy
  • Models, Molecular
  • Neuroimaging
  • Paraneoplastic Syndromes, Nervous System / immunology
  • Plasmapheresis
  • Protein Conformation
  • Psychosurgery
  • T-Lymphocytes, Cytotoxic / immunology

Substances

  • Autoantibodies
  • Autoantigens
  • Epitopes
  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents
  • Glutamate Decarboxylase
  • glutamate decarboxylase 2