Abstracts

Rationale:
Considering the dearth of literature on West syndrome(WS) from South Asian countries, this study aimed to evaluate the management practices in South Asia by means of an online-survey and meta-analysis.
Method:
An online questionnaire was sent to 223pediatric neurologists/pediatricians in India, Pakistan, Myanmar, SriLanka, Bhutan, Nepal, and Bangladesh. Their responses were evaluated and supplemented by a meta-analysis. Results Of 125responses received (Response-rate:56%),  around 60% of responders observed male preponderance and an approximate lead-time-to-treatment(LTTT) of 4-12 weeks. The commonest etiology observed was a static structural insult (88·6% of responders). Most commonly used first-line drug was(country-wise): India-adrenocorticotropin hormone(ACTH,50%); Pakistan-oral steroids(45·5%); Myanmar, Sri Lanka, and Nepal-oral steroids(94·4%); Bangladesh-ACTH(2/2); Bhutan- vigabatrin(3/5). ACTH and vigabatrin are not available in Myanmar and Nepal. The most commonly used regime for ACTH was maximal-dose-at-initiation-regime in India, Sri Lanka, and Bangladesh and gradually-escalating-regime in Pakistan. Maximum dose of prednisolone was variable- most common response from India:3-4mg/kg/day; Pakistan, Bhutan, and Bangladesh:2mg/kg/day; Sri Lanka, Nepal, and Myanmar:5-8mg/kg/day or 60mg/day. The total duration of hormonal therapy (including tapering) ranged from 4-12 weeks(67/91). Most responders considered cessation of spasms for four weeks as complete response(54/111) and advised electroencephalography(EEG;104/123) to check for hypsarrhythmia resolution. Difficult access to pediatric EEG in Bhutan and Nepal is concerning. More than 95% of responders felt a need for more awareness. The meta-analysis supported the preponderance of male gender(68%; confidence interval[CI]:64-73%), structural etiology(80%; CI 73-86%), longer LTTT(2·4months; CI 2·1-2·6 months), and low response-rate to hormonal therapy(18% and 28% for ACTH and oral steroids respectively) in WS in South Asia.
Conclusion:
This study highlights the practices and challenges in the management of WS in South Asia. These include a preponderance of male gender and structural etiology, a longer LTTT, difficult access to pediatric EEG, non-availability of ACTH and vigabatrin in some countries, and low effectiveness of hormonal therapy in this region.
Funding:
:None