Hypothalamic hamartomas and gelastic seizures are often associated with cognitive deterioration, behavioral problems, and poor response to anticonvulsant treatment or cortical resections. The origin and pathophysiology of the epileptic attacks are obscure. We investigated 3 patients with this syndrome and frequent gelastic seizures. Ictal single-photon emission computed tomography performed during typical gelastic seizures demonstrated hyperperfusion in the hamartomas, hypothalamic region, and thalamus without cortical or cerebellar hyperperfusion. Electroencephalographic recordings with depth electrodes implanted in the hamartoma demonstrated focal seizure origin from the hamartoma in 1 patient. Electrical stimulation studies reproduced the typical gelastic events. Stereotactic radiofrequency lesioning of the hamartoma resulted in seizure remission without complications 20 months after surgery. The functional imaging findings, electrophysiological data, and results of radiofrequency surgery indicate that epileptic seizures in this syndrome originate and propagate from the hypothalamic hamartoma and adjacent structures.

Dazuse polyspike discharge during sleep clinically related to dilateral jerking of the lips. motor seizures were reported. Interictal epileptiform abnormalities consisted of bursts of bilaterally synchronous sharp and slow wave discharges and independent sharp waves involving frontocentral and temporal regions. During sleep, fast recruiting rhythms and polyspike discharges were recorded, often related to a bilateral tonic contraction and/or jerking of the lips (Fig). In one patient these seizures recurred every few minutes for 3 hours. Additional history disclosed that similar seizures occurred even in the waking state. The lack of focal motor seizures in their patients was related by Kuzniecky and associates [ 11 to abnormal organization of the motor cortex. Our patients had similar electroclinical and neuroradiological findings, so that a different degree of structural abnormality leading to the occurrence of such a seizure type is unlikely. Focal motor seizures involving the lips were not initially mentioned and were mainly sleep related. Therefore, it is possible that Kuzniecky's patients also suffered from unrecognized oral motor seizures.Fast ictal discharges during sleep have been reported previousIy in severe focal or multifocal epilepsies [2, 37. Interestingly, the epileptic foci were mainly frontal and sudden falls were the predominant epileptic pattern 141.The bilateral motor ictal involvement of the mouth seems closely related to the opercular dysplasia and could be, if searched for, a further characteristic aspect of the epilepsy related to bilateral central macrogyria. In one of our patients oral seizures were so frequent during sleep that her dysarthria could have been aggravated by postictal cortical exhaustion. Neurological Institute