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

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.

Management of West syndrome during COVID-19 pandemic: A viewpoint from South Asian West Syndrome Research Group

“…South Asian West Syndrome Research Group (SAWSRG) was formed in early 2019, while studying the management practices for WS in South Asia ( Madaan et al, 2020 ). The members of the SAWSRG identified the key issues concerning COVID-19 with WS.…”

“…However, there would be applicability concerns for South Asia and developing countries due to peculiar management practices and challenges for WS. A preponderance of structural etiology, long treatment lag, difficult access to EEG, availability and licensing issues with the first-line drugs (ACTH and vigabatrin), and underdeveloped telehealth services make this region eccentric ( Madaan et al, 2020 ; Vaddi et al, 2018 ). Although the fundamental management principles are analogous, the global applicability of CNS recommendations is limited.…”

Financial Burden in Families of Children with West Syndrome

“…The first-line treatment options include adrenocorticotropic hormone (ACTH), oral steroids, and vigabatrin [ 4 ]. The short-term management goals are enduring cessation of epileptic spasms and resolution of hypsarrhythmia, which are achieved in nearly one-third of patients after first-line therapy [ 5 ]. The long-term neurodevelopmental outcome is often poor and depends on the initial therapeutic response [ 6 ].…”

“…Unlike the developed countries, the management of WS in India and developing countries has its own intricacies. These include a long treatment lag, preponderance of structural etiology, lack of awareness, and out-of-pocket expenditure for healthcare utilization [ 5 , 7 ]. Besides, two of the three first-line therapeutic options (ACTH and vigabatrin) are quite expensive, with ACTH often requiring hospitalization for the initiation and monitoring of adverse effects.…”

What Matters the Most for Hormonal Therapy in West Syndrome: Drug, Dosage, or Duration?

“…The treatment goals are enduring cessation of epileptic spasms and resolution of hypsarrhythmia. Hormonal therapy, in various forms [adrenocorticotropic hormone (natural, synthetic), oral steroids, intravenous pulse methylprednisolone] and dosage (high or low), has been used for its management with varying success [1]. Oral steroids are often preferred as the initial treatment considering the ease of administration and low-cost, especially during the coronavirus disease 2019 (COVID-19) pandemic [2].…”