Abstract

Recent advances in neuroimaging have allowed the detection and characterization of focal malformations of cortical developmental in a significant proportion of patients with epilepsy, many of whom were previously labelled as cryptogenic, allowing a better description of the associated electroencephalogram (EEG) features. Alpha activity is usually preserved, although superficial gyral abnormalities are often associated with overlying localized polymorphic delta activity, and occasionally abnormal fast activity. Most affected patients with epilepsy show interictal spikes. These are often broadly concordant with the structural abnormality but may show a wider anatomic distribution and be multifocal, or occasionally appear only in anatomically distant sites. In many patients the spikes are frequent and sometimes they occur continuously or in long trains. EEG findings are often stable over time, but some patients only show the development of slow wave changes or interictal spikes when followed serially for several years. A small proportion of patients with focal malformations of cortical development have EEG features mimicking idiopathic generalized epilepsy, and occasionally patients exhibit continuous generalized spike and slow wave activity in sleep. Electrocorticography studies confirm the often widespread nature of interictal spiking, but may also show highly epileptogenic patterns recorded directly from dysplastic cortex. The intrinsic epileptogenicity of areas of cortical developmental abnormalities has also been demonstrated by chronic intracranial studies and in vitro recordings of slices obtained from resected human dysplastic cortex. In this regard such developmental abnormalities are fundamentally different from acquired lesions such as tumors/vascular anomalies that usually exert their effects through changes in adjacent cortex.

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