Tuberous sclerosis, agenesis of the corpus callosum and Lennox-Gastaut syndrome: mere chance or a new syndrome?
The relationship between tuberous sclerosis (TS) and epilepsy on one hand and between agenesis of the corpus callosum (ACC) and epilepsy on the other has been recognized for a long time. Until now, a syndrome characterized by TS, ACC and Lennox-Gastaut syndrome (LGS) has not been described in the literature. Three cases of children, all young male patients, who suffer from TS, ACC and LGS, were presented. The ACC was total in two cases and partial in the third. None of the subjects previously had West syndrome. Two cases showed a slight degree of mental deficiency, while in the third it was very pronounced. In all subjects tonic and astatic seizures were frequent. Antiepileptic therapy improved the clinical picture in one case, while in the other two it remained unchanged.
DeMarco
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Department of Child Neurology, Centro Angeli Custodi, Istituti Ospedalieri, Trento, Italy.
D000293:Adolescent; D061085:Agenesis of Corpus Callosum; D001921:Brain / Q000000981:diagnostic imaging; D002648:Child; D003337:Corpus Callosum / Q000000981:diagnostic imaging; D004569:Electroencephalography; D004827:Epilepsy / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D008297:Male; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000150:complications* / Q000000981:diagnostic imaging