title: Infantile spasms in Down syndrome--effects of delayed anticonvulsive treatment.
abstract: To investigate the impact of treatment lag in infantile spasms (IS) on treatment response, occurrence of later epilepsy, and long-term cognition and behavior in patients with one single etiological entity, we examined 18 patients with Down syndrome (DS) and earlier IS retrospectively (follow-up period of 32-180 months with a mean of 85.1 months), and determined their history and present condition, in terms of previously mentioned items. There was a statistically significant correlation between treatment lag and lag to cessation of spasms (R=0.55, P=0.02), developmental quotient (DQ) (R=-0.75, P=0.003), and score of autistic features (AF) (R=0.57, P=0.04). Moreover we found that the later the response to treatment of IS, the lower was the DQ (R=-0.86, P=0.001) and the higher was the score of autistic features (R=0.5, P=0.06). A long duration of spasms also determined a low DQ (R=-0.93, P<0.0001) and a high score of autistic features (R=0.66, P<0.01). All patients with persistent epilepsy (n=5) had had a treatment lag of over 2 months. Conversely, for all children treated within 2 months (n=8) spasms ceased within 3 months of treatment and none of them had later epilepsy. This group of patients with a treatment lag of less than 2 months had earlier treatment response (P=0.002), higher DQ (P=0.004) and lower score of autistic features (P=0.006). The data stress the importance of a short treatment lag in view of mental development and prevention of later epilepsy and autistic features, and raise the question of antiepileptogenic effect in this specific condition.
authors.affiliation: Department of Neuropediatrics, Hôpital Saint Vincent de Paul, 82, Avenue Denfert Rochereau, F-75674 Paris cedex 14, France. M_Eisermann@compuserve.com
authors.forename: Monika Maria
authors.initials: MM
authors.lastname: Eisermann
authors.forename: A
authors.initials: A
authors.lastname: DeLaRaillère
authors.forename: G
authors.initials: G
authors.lastname: Dellatolas
authors.forename: E
authors.initials: E
authors.lastname: Tozzi
authors.forename: R
authors.initials: R
authors.lastname: Nabbout
authors.forename: O
authors.initials: O
authors.lastname: Dulac
authors.forename: C
authors.initials: C
authors.lastname: Chiron
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000008:administration & dosage*; D001321:Autistic Disorder / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications / Q000188:drug therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000150:complications / Q000188:drug therapy*; D018709:Statistics, Nonparametric; D013995:Time
chemical_list: D000927:Anticonvulsants