Autoimmunity to neuronal antigens is associated with seizures. These may poorly respond to standard antiseizures therapy.
Clinical features:
(1) seizure disorder
(2) variable cognitive dysfunction
(3) antibody to neuronal surface antigens
(4) variable evidence of autoimmune encephalitis
(5) variable autonomic dysfunction
(6) variable inflammatory CSF profile
(7) mesial temporal magnetic resonance imaging abnormalities
(8) no other better explanation
Some patients develop faciobrachial dystonic seizures with oral dyskinesia.
Some children have fever-induced refractory epileptic encephalopathy in school-aged children (FIRES).
Antigens targeted by autoantibodies:
(1) leucine-rich glioma inactivated 1 (LG1), which is associated with limbic encephalitis
(2) N-methyl-D-aspartate receptor (NMDAR) antibodies
(3) gamma-aminobutyric acid (GABA)
(4) alpha-amino-3-5-methylisoxazole-4-propionic acid (AMPA)
(5) glycine receptors
(6) glutamic acid decarboxylase 65 (GAD65)
Immunotherapy is often successful in reducing seizure frequency.
