Progress in autoimmune epileptic encephalitis


Purpose of review Autoimmune epileptic encephalopathy is a potentially treatable neurological syndrome characterized by the coexistence of a neuronal antibody in serum and, often, cerebrospinal fluid. Patients present with combinations of seizures, neuropsychiatric features, movement disorder, and cognitive decline, but some patients have isolated seizures either at first presentation or during their illness. This review summarizes our current understanding of the roles of specific neuronal antibodies in epilepsy-related syndromes and aims to aid the clinician in diagnosis and treatment. Recent findings Antigen discovery methods in three neuroimmunology centres independently identified antibodies to different subunits of the γ amino butyric acid-A receptor; high levels of these antibodies were found mainly in patients with severe refractory seizures. These and other antibodies were also found in a proportion (<10%) of children and adults with epilepsy. A clinical study comparing immunotherapy in patients with autoantibodies or without an identified target antigen found neuroinflammatory features were predictive of a therapeutic response. New in-vitro and in-vivo studies, and spontaneous animal models, have confirmed the pathogenicity and epileptogenicity of neuronal antibodies and their relevance to other mammals. Summary Neuronal antibodies are an important cause of autoimmune epileptic encephalopathy, early recognition is important as there may be an underlying tumour, and early treatment is associated with a better outcome. In the absence of an antibody, the clinician should adopt a pragmatic approach and consider a trial of immunotherapy when other causes have been excluded.

Publication DOI:
Divisions: College of Health & Life Sciences
Additional Information: This is the author's accepted manuscript of the following article: Progress in autoimmune epileptic encephalitis Wright, Sukhvir; Vincent, Angela. Current Opinion in Neurology: April 2016 - Volume 29 - Issue 2 - p 151–157. doi: 10.1097/WCO.0000000000000304
Uncontrolled Keywords: autoimmune encephalitis,autoimmune epilepsy,immunotherapy,neuronal surface antibodies,Neurology,Clinical Neurology
Publication ISSN: 1473-6551
Last Modified: 08 Jan 2024 08:55
Date Deposited: 04 Dec 2018 09:10
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Related URLs: http://www.scop ... tnerID=8YFLogxK (Scopus URL)
https://journal ... phalitis.8.aspx (Publisher URL)
PURE Output Type: Review article
Published Date: 2016-04-01
Accepted Date: 2016-01-01
Authors: Wright, Sukhvir (ORCID Profile 0000-0002-5464-3779)
Vincent, Angela



Version: Accepted Version

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