Spotlight on Antibody-mediated epilepsies

In this article, Dr. Sarosh Irani and Angela Vincent of Oxford University Hospitals and Dr. Bethan Lang of John Radcliffe Hospital summarize the recent advances in the field of antibody-mediated epilepsies. This entity began to be defined with the frequent observation of seizures in encephalitides associated with autoantibodies directed against surface antigens. The seizures in the antibody-positive cases are often best controlled with immunotherapies, which simultaneously reduce the levels of autoantibodies.

Recently, a new distinctive epileptic syndrome with frequent and brief dystonic episodes, typically involving the face and arm, has been recognized and found to be associated with VGKC-complex antibodies, almost always directed against LGI1. Patients with these “faciobrachial dystonic seizures” have a relatively poor response to antiepileptic drugs, but their seizures often respond better to immunotherapies. Emerging data suggest that patients who present with faciobrachial dystonic seizures progress to develop cognitive impairment, which may be prevented by treatment of the faciobrachial dystonic seizures. In addition, a few patients with status epilepticus and classical temporal lobe epilepsy have been described with these and other autoantibodies. Finally, although glutamic acid decarboxylase (GAD) is an intracellular enzyme, antibodies directed against GAD are found in patients with acute and chronic focal epilepsies and may be a marker of an autoimmune tendency. The antibody-mediated epilepsies are a growing field with important etiologic and therapeutic implications for both children and adults.

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