Abstracts

Rationale: ACTH, the preferred treatment for West Syndrome (WS) is expensive, requires several injections and hospitalization for days. Preliminary data suggest that MP may be a cheaper and effective alternative to ACTH. Objective: To compare the efficacy of MP with ACTH in children with WS Methods: Forty four newly diagnosed consecutive children between the ages of 3 months to 3 years with WS were included. Children with tuberous sclerosis or neurodegenerative disorders were excluded. A written informed consent was obtained. A detailed neurological examination and MRI were done in all cases. As ethical consideration did not permit randomization, all parents were offered the choice of either ACTH (synthetic ACTH 40 IU IM OD for 3 weeks, tapered over 3 weeks) or MP (30 mg/Kg/day as IV infusion for 5 days followed by oral prednisolone at 1 mg/Kg/day for 3 weeks and then tapered). Patients were monitored for cessation of spasms (using daily record of spasms by parents), blood pressure, random blood sugar, infection and weight gain. EEGs were obtained at enrolment and follow-up, and were scored (Bower and Jeavons method) by an independent neurologist. The primary outcome measure was cessation of spasms 6 weeks post enrolment and secondary outcome measures were reduction in number of spasms by 50% or more on day 14 of starting therapy, improvement in EEG score after 6 weeks and the frequency of adverse events. The study was approved by the Institute Ethics Committee. Results: There were 26 and 18 children in the ACTH and MP groups respectively; the two groups were similar with respect to mean age (ACTH-10.92 months, MP 11.86 months), socio demographic and seizure characteristics; 85% and 89% cases in the ACTH and MP groups respectively had symptomatic WS (p 0.52). One child in ACTH group expired prior to completion of therapy and one child in MP group was lost to follow-up. At two and at six weeks, there was no significant difference between the ACTH and MP groups with respect to complete cessation of spasms (10 vs 6, p 0.73 and 11 vs 4, p 0.17), reduction in number of spasms by >50% (15 vs 10, p 0.88 and 15 vs 13, p 0.27) and no reduction in number of spasms (8 vs 4, p 0.39 and 7 vs 4 p 0.75). The mean EEG scores at baseline (14.6+2.9 and 15.1+4.2), and after treatment (10.1+4.7 and 11.0+5.0, p 0.57), in the ACTH and MP groups were similar. No significant difference was found between the two groups for infection, weight gain, irritability and death. Hypertension was less common in MP as compared to ACTH group (2 vs 10 cases, p 0.046); hyperglycemia (n-4) was seen only in ACTH group. The cost of MP therapy was one sixth of that of ACTH therapy. Conclusions: Pulse MP followed by oral steroids achieved reduction in spasms and improvement in EEG that was comparable to ACTH. MP may be considered a less expensive alternative for treatment of West syndrome in resource poor situations.