Inherited epilepsy: spike-wave and focal motor seizures in the mutant mouse tottering

Abstract

Mice with the mutant gene tottering (tg, chromosome 8, autosomal recessive) show, in adolescence, abnormal bursts of bilaterally synchronous spike waves as revealed in electrocorticograms recorded over long periods. The spike waves are accompanied by behavioral "absence" attacks and intermittent focal motor seizures showing somatotopic progression. Cerebral metabolic activity during seizures was assayed by autoradiography of brain sections from mice injected intravenously with 14C-labeled 2-deoxyglucose. Metabolic activity was increased bilaterally in selected brainstem structures. Spontaneous electrocorticographic and clinical seizures of this general pattern were recognized hitherto only in humans.