Current Pediatric Research

Background and Aim: Vigabatrin (VGB) and Adrenocorticotropic Hormone (ACTH) are the two first line of treatment of Infantile Spasm (IS) in Tuberous Sclerosis Complex (TSC) patients. The aim of this study was to assess the efficacy and safety of these two drugs in TSC patients and to evaluate the EEG changes during treatment. Methods: We prospectively studied 22 consecutive infants with TSC and infantile spasm. Based on the initial therapy we divided the patients into two groups: Group-I: TSC who received VGB (n=15) and Group-II: TSC who received ACTH (n=7). The children who showed a partial or no response on treatment after 8 weeks, they were switched on another drug (VGB to ACTH and vice versa). The primary outcome was relapse of IS and secondary outcomes were peripheral visual field constriction, drowsiness, hypotonia, irritability, ear infection, hypertension, weight gain, irritability and stomach irritation. Results: Twenty-two TSC patients with IS were included. The median age of patients was 3 months (1-8 months) and 8 of them (36.8%) were females. At 12 months follow-up on initial therapy the frequency of complete responders (40% vs. 28.6%; p=0.02) and partial responders (33.3% vs. 14.3%; p=0.01) were higher and non-responders less prevalent (27.7% vs. 57.1%; p<0.001) in VGB arm compared to ACTH arm. Similarly, after switched on another drug, the non-responders to treatment were more prevalent in group switched on ACTH compared to group switched on VGB (50% vs. 25%; p=0.001). During follow up at 3, 6, 9 and 12 months the frequency of hypsarrhythmia was lower and multifocal activity was not significant compared to first recorded EEG in those two groups of treatment. In addition, at 12 months follow-up the hypsarrhythmia and multifocal activity were more prevalent in the group switched to VGB compared to the group switched on ACTH. Conclusions: Although refractory epilepsy is common, many patients achieve seizure control. This study suggests that the risk of infantile spasms relapse in TSC may be more reduced by vigabatrin treatment compared to ACTH.

Author(s): Naim Zeka, Ramush Bejiqi, Abdurrahim Gerguri, Ragip Retkoceri, Armend Vuciterna, Arlinda Maloku, Leonore Zogaj, Eris Zeka, Haki Jashari*

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