SIGMAR1 and encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy: Seizure disorders in Dravet syndrome mouse models caused by mutations in the Scn1a gene are accompanied by disturbances in GABAergic firing in hippocampal neurons [69], which may also indicate attenuation of epileptiform behavior due to Sigma1R-dependent modulation of GABAARs activity.