Classifying etiology of infantile spasms syndrome in resource‐limited settings: A study from the South Asian region

Wanigasinghe, Jithangi; Sahu, Jitendra Kumar; Madaan, Priyanka; Fatema, Kanij; Linn, Kyaw; Chand, Prem; Poudel, Prakash; Hamed, Esmatullah; Mynak, Mimi Lhamu; Hassan, Samaahath

doi:10.1002/epi4.12548

Cited by 11 publications

(13 citation statements)

References 27 publications

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“…Unlike all the previously reported children with DEE or infantile spasms (with genetic or presumed genetic etiology) with COVID-19, 3 of 5 children in the current study had underlying acquired structural etiology [5,18]. This concurs with a predominance of structural etiology in developing countries [19][20][21]. Also, the treatment lag (like previous studies) was huge in all 5 children, despite which, 3 had responded and were in remission [19,22].…”

Section: Discussioncontrasting

confidence: 45%

COVID-19 in Children with West Syndrome: An Ambispective Study

et al. 2022

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ObjectivesTo study the course of West syndrome (WS) and coronavirus disease-19 in children with WS who contracted SARS-CoV-2 infection. Methods This ambispective study was conducted at a tertiary-care center in North India between December 2020 and August 2021 after approval from the Institute Ethics Committee. Five children with WS, positive for COVID-19 based on RT-PCR, fulfilled the inclusion criteria. Results One child with COVID-19 during the first wave was retrospectively included while four children (of the 70 children screened) were prospectively enrolled. The median age at onset of epileptic spasms was 7 mo (2 boys), and that at presentation with COVID-19 was 18.5 mo. Three had underlying acquired structural etiology. Three were in remission following standard therapy, while two had ongoing spasms at the time of COVID-19 illness. During the illness, two of those in remission continued to be in remission while one child had a relapse. The children with ongoing epileptic spasms had variable course [one had persistent spasms and other had transient cessation lasting 3 wk from day 2 of COVID-19 illness, but electroencephalography (on day 8 of COVID-19 illness) continued to show hypsarrhythmia]. Fever was the most typical symptom (and sometimes the only symptom) of COVID-19, with a duration ranging from 1-8 d. Two children had moderate COVID-19 illness requiring hospitalization, while the rest had a mild illness. All the affected children had complete recovery from COVID-19. ConclusionThe severity of COVID-19 illness in children with WS is often mild, while the subsequent course of WS is variable.

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Section: Discussioncontrasting

confidence: 45%

COVID-19 in Children with West Syndrome: An Ambispective Study

et al. 2022

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“…For example, IESS due to hypoxic-ischemic injury is reported in 58% in Bangladesh, 34% in India, 50% in Nepal, 32% in Pakistan, 28% in Myanmar, and 20% in Sri Lanka. 6,16 In our study, this proportion increased to 39%. These figures contrast with the 10% reported in patients in the United Kingdom Infantile Spasms Study (UKISS).…”

Section: Discussionsupporting

confidence: 42%

“…They are reported in higher proportions in countries in the low‐income strata. For example, IESS due to hypoxic‐ischemic injury is reported in 58% in Bangladesh, 34% in India, 50% in Nepal, 32% in Pakistan, 28% in Myanmar, and 20% in Sri Lanka 6,16 . In our study, this proportion increased to 39%.…”

Section: Discussionsupporting

confidence: 41%

“…The latter is common in resource‐limited settings, mainly due to the limitations in availability and accessibility to the diagnostic studies needed to assess structural, metabolic, and genetic etiologies. As previously suggested by investigators from the South Asian region, this group of children can be categorized as “etiology incompletely evaluated.” 6 Nonavailability of consecutive samples across the participating sites may have bias towards inclusion of children who are more likely to respond to therapy. Despite these limitations, the relatively large sample size has enabled us to derive several useful observations on etiology and response to therapy and factors to be improved in the management of IESS.…”

Section: Discussionmentioning

confidence: 99%

“…Considering the wide range of etiologies of IESS, regional differences in their prevalence are very likely, for example, the differences in the proportions of etiological subtypes reported from the United States 3 and those reported from India 5 . Understanding these regional differences 6 is crucial since etiology is the single most important factor associated with better prognosis 7 . The absence of a known etiology is associated with better cognitive outcomes as shown in several studies 8 .…”

Section: Introductionmentioning

confidence: 99%

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Demographic characteristics and clinical presentation of infants with infantile epileptic spasms syndrome and their response to therapy: Data from Sri Lanka Infantile Spasms Registry

Wanigasinghe

Hewawitharana

Ratnayake

et al. 2023

Annals of the Child Neurology Society

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Objectives Infantile epileptic spasm syndrome (IESS) is an epileptic encephalopathy with often devastating developmental consequences. Most children with IESS have a known etiology, although differing in proportion by geographical settings. Therefore, registries are useful to understand the characteristics of IESS in different countries. The Sri Lanka Infantile Spasms Registry (SLISR) was established to study the demographics and etiology of infants with IESS and their response to therapy in a resource‐limited country. Methods Five pediatric neurologists (out of nine) in different parts of the country prospectively recruited children with IESS. The etiology was evaluated using the services available in each setting. Response to treatment for standard (adrenal corticotropic hormone, prednisolone, or vigabatrin) versus nonstandard medications was evaluated at two and six weeks. Results Included in the current analysis were 270 children who were registered since 2017. Median age at presentation was 5.36 months (SD 3.6). The mean interval between seizure onset and treatment onset was 1.7 months (SD 1.3). A sizable proportion of the children (61.2%) did not complete the evaluation of etiology. Structural brain abnormality was the most frequently identified etiology in those who were evaluated (38.8%); hypoxic‐ischemic injury was the most common antecedent. The majority of the patients (86%) received a recommended standard therapy as the first treatment, with prednisolone being the most frequent choice. By treatment day 14, the first treatment had achieved spasm control in 63.8% and an electro‐clinical response in 43.6%. While both standard therapies led to positive outcomes, oral prednisolone produced the best therapeutic response. Conclusion We describe the etiologies, treatment choices, and response to first‐line medications in a large group of children with IESS from a South Asian country. Although most patients received a recommended first‐line therapy (most often prednisolone), a sizable number initially received nonstandard therapy. Our data illustrate the challenges in the management of IESS in a resource‐limited environment.

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