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Peer-reviewedCC BYCureus

Long-Term Outcome of West Syndrome Treated With Adrenocorticotropic Hormone-Based Therapy in a Tertiary Care Hospital in Bangladesh

5 affiliations
  1. 1Department of Pediatric Neurology, National Institute of Neurosciences & Hospital, Dhaka, BGD
  2. 2Department of Neurophysiology, National Institute of Neurosciences & Hospital, Dhaka, BGD
  3. 3Department of Colorectal Surgery, Dhaka Medical College & Hospital, Dhaka, BGD
  4. 4Department of Pediatric Neurology, Khulna Medical College & Hospital, Khulna, BGD
  5. 5Department of Neurology, National Institute of Neurosciences & Hospital, Dhaka, BGD

Abstract

Background West syndrome is a severe developmental and epileptic encephalopathy associated with epileptic spasms, abnormal EEG findings, and poor long-term neurodevelopmental outcomes. Adrenocorticotropic hormone (ACTH)-based therapies are widely used for treatment, but data on seizure, EEG, and developmental outcomes from Bangladesh remain limited. Objective The aim of this study was to evaluate seizure outcomes, EEG changes, and long-term functional developmental outcomes among children with West syndrome treated with different ACTH-based regimens in a tertiary care hospital in Bangladesh. Methods This retrospective observational follow-up study included 50 children with West syndrome treated at the Department of Pediatric Neurology, National Institute of Neurosciences & Hospital, Dhaka, Bangladesh, between 2018 and 2024. Children were categorized into three treatment groups: high-dose ACTH, n = 18; combination therapy with high-dose ACTH plus vigabatrin, n = 10; and medium-dose ACTH, n = 22. Seizure outcomes, post-treatment EEG findings, and long-term functional developmental outcomes were assessed from clinical records, EEG reports, caregiver interviews, and structured parental surveys. Data were analyzed using SPSS Version 22.0, with p < 0.05 considered statistically significant. Results Baseline age distribution, age at onset of spasms, and sex did not differ significantly among treatment groups. Seizure outcome differed significantly across groups, with seizure freedom observed in 61.1% of children in the high-dose ACTH group, 50.0% in the combination therapy group, and 4.5% in the medium-dose ACTH group. Lennox-Gastaut syndrome was most frequent in the medium-dose ACTH group, 72.7%. EEG normalization was highest in the high-dose ACTH group, 44.4%, followed by the combination therapy group, 20.0%, and the medium-dose ACTH group, 13.6%, although this difference was not statistically significant. Developmental outcome also did not differ significantly among groups; however, dependent living was most frequent in the medium-dose ACTH group, 59.1%. Conclusion High-dose ACTH was associated with better seizure freedom and a higher proportion of EEG normalization in this cohort, while medium-dose ACTH was associated with poorer seizure outcome, more frequent Lennox-Gastaut syndrome, and greater developmental dependency. However, the retrospective non-randomized design and small sample size limit causal interpretation. Larger prospective studies with standardized treatment protocols, etiological classification, relapse assessment, adverse-effect monitoring, and validated developmental evaluation are needed.

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BibTeX
@article{sumi2026longterm,
  title = {{Long-Term Outcome of West Syndrome Treated With Adrenocorticotropic Hormone-Based Therapy in a Tertiary Care Hospital in Bangladesh}},
  author = {Samsun N Sumi and Abdullah A Mamun and Sufia K Sumi and Sarah Alam and Ahsan Habib and Mosiur Rahman and Nazmul Haque and Tuhfie Jannat and Yamin Shahriar Chowdhury and Rajib N Chowdhury},
  journal = {Cureus},
  year = {2026},
  month = sep,
  doi = {10.7759/cureus.117115},
  url = {https://europepmc.org/article/PMC/PMC13717257},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - Long-Term Outcome of West Syndrome Treated With Adrenocorticotropic Hormone-Based Therapy in a Tertiary Care Hospital in Bangladesh
AU  - Samsun N Sumi
AU  - Abdullah A Mamun
AU  - Sufia K Sumi
AU  - Sarah Alam
AU  - Ahsan Habib
AU  - Mosiur Rahman
AU  - Nazmul Haque
AU  - Tuhfie Jannat
AU  - Yamin Shahriar Chowdhury
AU  - Rajib N Chowdhury
PY  - 2026
DA  - 2026/09/28
JO  - Cureus
DO  - 10.7759/cureus.117115
UR  - https://europepmc.org/article/PMC/PMC13717257
AB  - Background West syndrome is a severe developmental and epileptic encephalopathy associated with epileptic spasms, abnormal EEG findings, and poor long-term neurodevelopmental outcomes. Adrenocorticotropic hormone (ACTH)-based therapies are widely used for treatment, but data on seizure, EEG, and developmental outcomes from Bangladesh remain limited. Objective The aim of this study was to evaluate seizure outcomes, EEG changes, and long-term functional developmental outcomes among children with West syndrome treated with different ACTH-based regimens in a tertiary care hospital in Bangladesh. Methods This retrospective observational follow-up study included 50 children with West syndrome treated at the Department of Pediatric Neurology, National Institute of Neurosciences & Hospital, Dhaka, Bangladesh, between 2018 and 2024. Children were categorized into three treatment groups: high-dose ACTH, n = 18; combination therapy with high-dose ACTH plus vigabatrin, n = 10; and medium-dose ACTH, n = 22. Seizure outcomes, post-treatment EEG findings, and long-term functional developmental outcomes were assessed from clinical records, EEG reports, caregiver interviews, and structured parental surveys. Data were analyzed using SPSS Version 22.0, with p < 0.05 considered statistically significant. Results Baseline age distribution, age at onset of spasms, and sex did not differ significantly among treatment groups. Seizure outcome differed significantly across groups, with seizure freedom observed in 61.1% of children in the high-dose ACTH group, 50.0% in the combination therapy group, and 4.5% in the medium-dose ACTH group. Lennox-Gastaut syndrome was most frequent in the medium-dose ACTH group, 72.7%. EEG normalization was highest in the high-dose ACTH group, 44.4%, followed by the combination therapy group, 20.0%, and the medium-dose ACTH group, 13.6%, although this difference was not statistically significant. Developmental outcome also did not differ significantly among groups; however, dependent living was most frequent in the medium-dose ACTH group, 59.1%. Conclusion High-dose ACTH was associated with better seizure freedom and a higher proportion of EEG normalization in this cohort, while medium-dose ACTH was associated with poorer seizure outcome, more frequent Lennox-Gastaut syndrome, and greater developmental dependency. However, the retrospective non-randomized design and small sample size limit causal interpretation. Larger prospective studies with standardized treatment protocols, etiological classification, relapse assessment, adverse-effect monitoring, and validated developmental evaluation are needed.
SN  - 2168-8184
ER  -