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DOI: 10.1177/000992289203100804 Gelastic EpilepsyA Clinical ContributionDepartment of Pediatrics, Division of Child Neurology
Department of Experimental Medicine, Chair of Medical Genetics
Department of Pediatrics, Division of Child Neurology
Department of Pediatrics, Division of Child Neurology
Department of Neurologic Science, Chair of Neuroradiology University "La Sapienza" Rome, Italy
Department of Neurologic Science, Chair of Neuroradiology University "La Sapienza" Rome, Italy Gelastic (laughing) epilepsy, relatively uncommon, is usually associated with hypothalamic hamartomas, pituitary tumors, astrocytomas of the mammillary bodies, and dysraphic conditions. Cases of unknown etiology are rare. In three of the four cases reported here, the diagnoses were hamartoma of the tuber cinereum; lobar holoprosencephaly; and lissencephaly type I, grade 2. In the fourth, radiographic investigation gave a normal result; a genetic etiology was suggested because of bilateral familial idiopathic epilepsy. In all patients, EEGs showed both focal spikes and generalized spike-and-wave discharges. The primary underlying neurophysiologic disorder may be provoked by the diffuse hyperexcitability of the cortex and subsequent firing of the thalamocortical networks with which the cortical brain is reciprocally interlinked.
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