Antiglutamic acid decarboxylase 65 (GAD65) antibody-associated epilepsy

Ahmad Daif, Rimas Vincas Lukas, Naoum P. Issa, Adil Javed, Stephen Anthony VanHaerents, Anthony T. Reder, James X. Tao, Peter Warnke, Sandra Rose, Vernon L. Towle, Shasha Wu*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

37 Scopus citations

Abstract

Glutamic acid decarboxylase (GAD) antibody-associated encephalitis causes both acute seizures and chronic epilepsy with predominantly temporal lobe onset. This condition is challenging in diagnosis and management, and the incidence of GAD antibody (Ab)-related epilepsy could be much higher than commonly believed. Imaging and CSF evidence of inflammation along with typical clinical presentations, such as adult onset temporal lobe epilepsy (TLE) with unexplained etiology, should prompt testing for the diagnostic antibodies. High serum GAD Ab titer (≥ 2000 U/mL or ≥ 20 nmol/L) and evidence of intrathecal anti-GAD Ab synthesis support the diagnosis. Unlike other immune-mediated epilepsies, antiglutamic acid decarboxylase 65 (GAD65) antibody-mediated epilepsy is often poorly responsive to antiepileptic drugs (AEDs) and only moderately responsive to immune therapy with steroids, intravenous immunoglobulin (IVIG), or plasma exchange (PLEX). Long-term treatment with more aggressive immunosuppressants such as rituximab (RTX) and/or cyclophosphamide is often necessary and may be more effective than current immunosuppressive approaches. The aim of this review is to review the physiology, pathology, clinical presentation, related ancillary tests, and management of GAD Ab-associated autoimmune epilepsy by searching the keywords and to promote the recognition and the initiation of proper therapy for this condition.

Original languageEnglish (US)
Pages (from-to)331-336
Number of pages6
JournalEpilepsy and Behavior
Volume80
DOIs
StatePublished - Mar 1 2018

Keywords

  • Autoimmune encephalitis
  • Autoimmune epilepsy
  • Limbic encephalitis
  • Paraneoplastic limbic encephalitis

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology
  • Behavioral Neuroscience

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