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Autoimmune Epilepsies 

Autoimmune Epilepsies
Chapter:
Autoimmune Epilepsies
Author(s):

Sarosh R. Irani

, Christian G. Bien

, and Angela Vincent

DOI:
10.1093/med/9780199796793.003.0015
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date: 20 January 2022

In the last decade, serum autoantibodies have been identified that are likely to be pathogenic in many cases of epilepsy. Established antibody targets include membrane or membrane-associated neuronal proteins such as components of the voltage-gated potassium channel complex (LGI1, CASPR2 and Contactin-2) and the NMDA, GABAB and AMPA receptors. Many of the ‘autoimmune encephalitides’ associated with these antibodies include seizures as a prominent part of the syndrome, but commonly also present with amnesia and confusion. The seizures and cognitive deficits usually show marked improvements with immunotherapies, and their amelioration is well correlated with reductions in antibody levels in individual patients. Antibodies to VGKC-complexes or GAD have also been described in cohorts of patients with various forms of epilepsy, although the etiological role of these antibodies is not yet clear. Recently, a new seizure semiology has been described that is closely associated with the presence of LGI1-antibodies. Very frequent short-lived, adult-onset arm and face dystonic spasms, termed faciobrachial dystonic seizures (FBDS), were first identified within the context of LGI1-antibody associated limbic encephalitis (LE) but most cases precede LE. FBDS respond well to immunotherapies but relatively poorly to antiepileptic drugs. FBDS provide the first example of a pure epilepsy syndrome which is strongly associated with autoantibodies and a good response to immunotherapies. The field of autoimmune epilepsies is gathering pace and there are likely to be further developments in the next few years.

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