Clinical profile and treatment of infantile spasms using vigabatrin and ACTH - a developing country perspective

Shahnaz Ibrahim,Taimur Saleem,Sidra Ishaque,Shamshad Gulab

doi:10.1186/1471-2431-10-1

Abstract

BackgroundInfantile spasms represent a serious epileptic syndrome that occurs in the early infantile age. ACTH and Vigabatrin are actively investigated drugs in its treatment. This study describes the comparison of their efficacy in a large series of patients with infantile spasms from Pakistan.MethodsAll patients with infantile spasms who presented to Aga Khan University Hospital, Karachi, Pakistan from January, 2006 to April, 2008 were included in this study. Inclusion criteria were clinical symptoms of infantile spasms, hypsarrythmia or modified hyparrythmia on electroencephalography, at least six months of follow-up period and receipt of any of the two drugs mentioned above. The type of drug distribution was random according to the availability, cost and ease of administration.ResultsFifty six cases fulfilled the inclusion criteria. 62.5% were males. Mean age at onset of seizures was 5 ± 1.4 months. Fifty two (92.8%) patients demonstrated hypsarrythmia on electroencephalography. 64.3% cases were identified as symptomatic while 19.6% were cryptogenic and 16.1% were idiopathic. Eighteen patients received ACTH while 38 patients received Vigabatrin as first line therapy. Initial response to first line therapy was similar (50% for ACTH and 55.3% for Vigabatrin). Overall, the symptomatic and idiopathic groups responded better to Vigabatrin. The relapse rate was higher for ACTH as compared to Vigabatrin (55.5% vs. 33.3%) when considering the first line therapy. Four patients evolved to Lennox-Gastaut variant; all of these patients had initially received Vigabatrin and then ACTH.ConclusionVigabatrin and ACTH showed no significant difference in the initial treatment of infantile spasms. However, patients receiving ACTH were 1.2 times more likely to relapse as compared to the patients receiving Vigabatrin when considering monotherapy. We suggest that Vigabatrin should be the initial drug of choice in patients presenting with infantile spasms. However, larger studies from developing countries are required to validate the therapeutic trends observed in this study.

Highlights

Infantile spasms represent a serious epileptic syndrome that occurs in the early infantile age
Cerebral palsy secondary to birth asphyxia was identified as the leading cause of infantile spasms (Table 2)
It was observed that the patients started on adrenocorticotropic hormone (ACTH) had earlier relapses and delayed recovery, as opposed to those started on Vigabatrin therapy. These time durations were 2 weeks for the former and 2 months for the later. Those who were switched to Vigabatrin after prior ACTH therapy relapsed after 6 months on average as opposed to those who were switched to ACTH therapy after prior Vigabatrin therapy, who relapsed after an average time of 2 months. (Table 5) Patients receiving ACTH were 1.2 times more likely to relapse as compared to the patients receiving Vigabatrin when considering monotherapy. d

Summary

Introduction

Infantile spasms represent a serious epileptic syndrome that occurs in the early infantile age. Infantile spasms represent a unique and age-specific epilepsy disorder usually presenting in early infancy. Infantile spasms are associated with devastating neurological results. This severe epilepsy syndrome is often considered an age-dependent expression of a damaged brain [1]. Infantile spasm may be seen either alone or in the constellation of West Syndrome. West syndromee consists of the triad of infantile spasms, an interictal EEG pattern (hypsarrhythmia or similar pattern), and mental retardation. The key features to diagnosing infantile spasms are patient history, age, characteristic semiology of seizures, and distinctive EEG pattern [3]

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