Abstract

Aims West Syndrome is an age dependent epileptic encephalopathy with variable therapeutic response. Worldwide, there is scarce data on magnitude and effect of treatment lag on therapeutic response with adrenocorticotrophic hormone (ACTH) or steroid therapy in West Syndrome. There is conflicting evidence regarding impact of treatment lag in West Syndrome. The present study aimed to elucidate magnitude of diagnostic and treatment lag, potential determinants of treatment lag and its impact on short-term therapeutic response with ACTH or oral steroids therapy. Methods We conducted a prospective study in a tertiary care hospital in northern India between January-December 2014. A total of 82 consecutive children with West Syndrome were enrolled. Magnitude and determinants of treatment lag were determined in all the children. Parents were interviewed and medical records of the child were reviewed. The treatment lag was calculated as the time delay between onset of spasms and initiation of ACTH/oral steroids treatment. Short term therapeutic response was taken as cessation of spasms within 14 days of therapy and sustained for a period of 28 days from the last witnessed spasm. We analysed following potential predictors: age of onset of spasms, aetiology, treatment lag and gender for their association with short-term therapeutic response. Results The median treatment lag duration was 90 days (95% CI: 110–198 days). The significant determinants of treatment lag in our study were: the pre-existing delay of children, educational status of the parents and qualification of the first practitioner visited. Our study showed shorter treatment lag ( Conclusion We observed a significant treatment lag in our children with West Syndrome. The lead time to treatment emerged as a potential modifiable risk factor for therapeutic response with ACTH/steroid therapy.

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