Gelastic epilepsy. A clinical contribution.
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.
Iannetti
P
P
Department of Pediatrics, University La Sapienza Rome, Italy.
Chessa
L
L
Raucci
U
U
Basile
L A
LA
Fantozzi
L M
LM
Bozzao
L
L
D000293:Adolescent; D002540:Cerebral Cortex / Q000002:abnormalities; D002648:Child; D004569:Electroencephalography; D004827:Epilepsy / Q000145:classification / Q000175:diagnosis* / Q000209:etiology; D005260:Female; D006222:Hamartoma / Q000150:complications / Q000175:diagnosis; D016142:Holoprosencephaly / Q000150:complications; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000150:complications / Q000175:diagnosis; D007845:Laughter; D008279:Magnetic Resonance Imaging; D008297:Male; D013239:Stereotyped Behavior; D014057:Tomography, X-Ray Computed; D014371:Tuber Cinereum