Focal dysplasia of the cerebral cortex in epilepsy

An unusual microscopic abnormality has been identified in the lobectomy specimens removed surgically from the brains of 10 epileptic patients. The abnormality could seldom be identified by palpation or with the naked eye. Histologically, it consisted of congregations of large, bizarre neurones which were littered through all but the first cortical layer. In most, but not in all cases, grotesque cells, probably of glial origin, were also present in the depths of the affected cortex and in the subjacent white matter. This kind of abnormality appears to be a malformation. The picture is reminiscent of tuberous sclerosis but too many distinguishing features, both in the clinical and in the pathological aspects, make this diagnosis untenable. The cases are therefore looked on provisionally (since all but one are still alive) as comprising a distinct form of cortical dysplasia in which localized, exotic populations of nerve cells underlie the electrical and clinical manifestations of certain focal forms of epilepsy.