Autoimmune epilepsy

Based on recent studies, a considerable minority of patients with epilepsy of unknown etiology may have an autoimmune or paraneoplastic cause. Autoimmune seizures or epilepsy usually presents with a subacute onset of refractory seizures along with cognitive, behavioral, or psychiatric dysfunction. Leucine-rich glioma protein1 (LGI-1), N-methyl-D-aspartate receptor (NMDA-R), and glutamic acid decarboxylase 65 (GAD 65) are the most common immunoglobulin G (IgG) specificities associated with autoimmune seizures or epilepsy. Diagnosis of these cases depends on the identification of the clinical syndrome, ancillary studies, and autoantibody testing. Predictive models based on clinical features and initial neurological assessment (antibody prevalence in epilepsy and encephalopathy [APE2] and response to immunotherapy in epilepsy and encephalopathy [RITE2] scores) may be utilized for selection of cases for autoimmune epilepsy evaluation and management. Early initiation of immunotherapy is critical for favorable clinical outcomes. Utilization of immunotherapeutic agents for autoimmune epilepsy management is divided into first-line and second-line treatments. These are incorporated into a proposed treatment algorithm. In the ensuing years, the field of autoimmune epilepsy is likely to expand further with discovery of several novel autoantibodies and improved mechanistic understanding.