title: Mental and behavioural outcome of infantile epilepsy treated by vigabatrin in tuberous sclerosis patients.
abstract: Vigabatrin (VGB) has demonstrated high efficacy in infantile spasms (IS) due to tuberous sclerosis. Our first objective was to evaluate the cognitive long term effect outcome of children whose refractory spasms definitely disappeared when VGB was given as an add on drug. Our second objective was to determine the response of generalized epilepsy (infantile spasms) compared to partial epilepsy on cognitive impairment. A non selected series of 13 children underwent psychometric and behavioural evaluation before VGB initiation at a mean of 3 years on VGB treatment. Eight of them could perform detailed neuropsychological tests at follow-up. Seven had infantile spasms (Group I), they all were spasm free before 2 years of age and five remained with rare partial seizures (mean age, 5.5 years). Six others had partial epilepsy without spasms (Group II) and five remained with rare seizures (mean age, 7.5 years). Patients of Group I experienced dramatic changes. Developmental quotient (DQ) significantly rose in six out of seven by ten to more than 45 points (P = 0.03) and autistic behaviour disappeared in five out of the six who presented with. The four tested children had normal verbal level after 5 years and could integrate at school but they remained with marked visuospatial disabilities. By contrast, patients of Group II remained with an unchanged DQ of about 60 so that both groups had similar DQ levels on follow-up. The cessation of spasms with VGB is therefore associated with significant improvement of cognition and behaviour in children with tuberous sclerosis. Controlling secondary generalization induced by infantile spasms seems to be a key factor for mental development.
authors.affiliation: Hospital Saint Vincent de Paul, Department of Pediatric Neurology, Paris, France.
authors.forename: I
authors.initials: I
authors.lastname: Jambaqué
authors.forename: C
authors.initials: C
authors.lastname: Chiron
authors.forename: C
authors.initials: C
authors.lastname: Dumas
authors.forename: J
authors.initials: J
authors.lastname: Mumford
authors.forename: O
authors.initials: O
authors.lastname: Dulac
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D001321:Autistic Disorder / Q000209:etiology; D002653:Child Behavior Disorders / Q000209:etiology*; D003072:Cognition Disorders / Q000209:etiology*; D004359:Drug Therapy, Combination; D004828:Epilepsies, Partial / Q000188:drug therapy* / Q000209:etiology / Q000523:psychology; D004829:Epilepsy, Generalized / Q000188:drug therapy / Q000209:etiology / Q000517:prevention & control / Q000523:psychology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006948:Hyperkinesis / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000209:etiology*; D007361:Intelligence Tests; D007859:Learning Disabilities / Q000209:etiology; D008297:Male; D009483:Neuropsychological Tests; D011597:Psychomotor Performance; D013036:Spasms, Infantile / Q000188:drug therapy* / Q000209:etiology / Q000523:psychology; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000150:complications*; D020888:Vigabatrin / Q000627:therapeutic use*
chemical_list: D000927:Anticonvulsants; D020888:Vigabatrin