Management practices for West syndrome in South Asia: A survey study and meta-analysis

Priyanka Madaan; Prem Chand; Kyaw Linn; Jithangi Wanigasinghe; Mimi Lhamu Mynak; Prakash Poudel; Raili Riikonen; Amit Kumar; Pooja Dhir; Sandeep Negi; Jitendra Kumar Sahu

doi:10.1002/epi4.12419

Management practices for West syndrome in South Asia: A survey study and meta-analysis

Epilepsia Open. 2020 Aug 11;5(3):461-474. doi: 10.1002/epi4.12419. eCollection 2020 Sep.

Authors

Affiliations

¹ Pediatric Neurology Unit Department of Pediatrics Postgraduate Institute of Medical Education and Research Chandigarh India.
² Aga Khan University Karachi Pakistan.
³ Pediatric Neurology Unit Yangon Children Hospital Yangon Myanmar.
⁴ Department of Paediatrics University of Colombo Colombo Sri Lanka.
⁵ Department of Pediatrics Jigme Dorji Wangchuck National Referral Hospital Thimphu Bhutan.
⁶ Department of Pediatrics B.P. Koirala Institute of Health Sciences Dharan Nepal.
⁷ Child Neurology Children's Hospital University of Eastern Finland and Kuopio University Hospital Kuopio Finland.
⁸ Department of Neurology All India Institute of Medical Sciences New Delhi India.

Abstract

Objectives: 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 an online survey and meta-analysis.

Methods: An online questionnaire was sent to 223 pediatric neurologists/pediatricians in India, Pakistan, Myanmar, Sri Lanka, Bhutan, Nepal, and Bangladesh. Their responses were evaluated and supplemented by a meta-analysis.

Results: Of 125 responses 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 (country-wise) was as follows: 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: 3-4 mg/kg/d; Pakistan, Bhutan, and Bangladesh: 2 mg/kg/d; Sri Lanka, Nepal, and Myanmar: 5-8 mg/kg/d or 60 mg/d. The total duration of hormonal therapy (including tapering) ranged from 4 to 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 (2.4 months; 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.

Significance: 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, nonavailability of ACTH and vigabatrin in some countries, and low effectiveness of hormonal therapy in this region.

Keywords: Asia; epileptic spasms; hypsarrhythmia; infantile spasms; low middle‐income countries.