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The recognition and treatment of autoimmune epilepsy in children
Author(s) -
Suleiman Jehan,
Dale Russell C
Publication year - 2015
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.12647
Subject(s) - autoimmune encephalitis , epilepsy , medicine , immunology , encephalitis , limbic encephalitis , autoantibody , context (archaeology) , antibody , biology , psychiatry , paleontology , virus
There is emerging interest in autoimmune epilepsy, which represents a small but potentially treatable form of epilepsy. Most insights into autoimmune epilepsy derive from the recent descriptions of autoimmune encephalitis that takes two general forms: a focal encephalitis (such as limbic) or a diffuse encephalitis (such as anti‐ N ‐methyl‐ d ‐aspartate receptor [ NMDAR ] encephalitis). The features of autoimmune epilepsy include acute or subacute onset of seizures, usually in the context of encephalopathy, and inflammation of the central nervous system on testing cerebrospinal fluid or magnetic resonance imaging. Neuronal antibodies associated with autoimmune encephalitis and seizures in children include NMDAR , voltage‐gated potassium channel complex, glycine receptor, γ ‐Aminobutyric acid type A receptor ( GABA A R ), γ ‐Aminobutyric acid type B receptor ( GABA B R ), and glutamic acid decarboxylase antibodies. These antibodies support the diagnosis of autoimmune epilepsy, but are not essential for diagnosis. When autoimmune epilepsy is suspected, first‐line immune therapy with corticosteroids in addition to intravenous immunoglobulin or plasma exchange should be considered. Second‐line therapy with rituximab or cyclophosphamide can be considered if the syndrome is severe. A response to immune therapy supports the diagnosis of autoimmune epilepsy. Neuronal antibodies are increasingly found in patients with focal epilepsy of unknown cause who do not have ‘encephalitis’. Recent epidemiological studies support the link between epilepsy and autoimmune diseases. Future studies need to define the spectrum of autoimmune epilepsy and focus on early identification and treatment.

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