Current status of treatment for infantile epileptic spasm syndrome_Journal of Epilepsy

Authors：

ZHAO Kaoming , YI Zhaoshi ,  ZHONG Jianmin

Department of Neurology, Jiangxi Children's Hospital, NanChang 330006, China;

Corresponding author：

ZHONG Jianmin, Email: zhongjm@163.com

Keywords：

Infantile epileptic spasm syndrome; Treatment; Prognosis

DOI：

10.7507/2096-0247.202405002

Video：

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Infantile epileptic spasm syndrome (IESS) is an age-dependent epileptic encephalopathy that onset within 3 ~ 12 months. Commonly used Anti-seizure medications (ASMs) have poor efficacy, low long-term control rate and high recurrence rate, and often leave behind sequelae such as developmental delay/intellectual disability, and autism spectrum disorders. At present, the first-line recommended treatments for IESS are Adrenocorticotropic hormone (ACTH), adrenocortical hormone and vigabatrin. If ineffective, other ASMs, ketogenic diets, and surgical treatments can be tried. This article will provide a review of the current status and related clinical applications of IESS treatment.

Citation： ZHAO Kaoming, YI Zhaoshi, ZHONG Jianmin. Current status of treatment for infantile epileptic spasm syndrome. Journal of Epilepsy, 2024, 10(4): 332-339. doi: 10.7507/2096-0247.202405002 Copy

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16. Duchowny MS, Chopra I, Niewoehner J, et al. A systematic literature review and indirect treatment comparison of efficacy of repository corticotropin injection versus synthetic adrenocorticotropic hormone for infantile spasms. J Health Econ Outcomes Res, 2021, 8(1): 1-9.
17. Zhu HM, Yuan CH, Luo MQ, et al. Safety and effectiveness of oral methylprednisolone therapy in comparison with intramuscular adrenocorticotropic hormone and oral prednisolone in children with infantile spasms. Front Neurol, 2021, 12: 756746.
18. Osborne JP, Edwards SW, Alber FD, et al. Prednisolone or tetracosactide depot for infantile epileptic spasms syndrome? A prospective analysis of data embedded within two randomised controlled trials. Eur J Paediatr Neurol, 2023, 42: 110-116.
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22. Shao LR, Stafstrom CE. Pediatric epileptic encephalopathies: pathophysiology and animal models. Semin Pediatr Neurol, 2016, 23(2): 98-107.
23. Hancock E, Osborne J. Treatment of infantile spasms with high-dose oral prednisolone. Dev Med Child Neurol, 1998, 40(7): 500.
24. Basit A, Noreen N, Saleem SF, et al. Comparison of efficacy and safety of low- versus high-dose oral prednisolone in infantile spasm (IS): an open label randomized controlled trial at the Children's Hospital & Institute of Child Health, Multan, Pakistan. Cureus, 2022, 14(3): e23164.
25. Lux AL, Edwards SW, Hancock E, et al. The United Kingdom infantile spasms study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomised controlled trial. Lancet, 2004, 364(9447): 1773-1778.
26. O'Callaghan FJ, Edwards SW, Alber FD, et al. Safety and effectiveness of hormonal treatment versus hormonal treatment with vigabatrin for infantile spasms (ICISS): a randomised, multicentre, open-label trial. Lancet Neurol, 2017, 16(1): 33-42.
27. 陈辉, 钟建民, 易招师, 等. 泼尼松治疗婴儿痉挛症的免疫机制研究. 中国当代儿科杂志, 2017, 19(10): 1044-1050.
28. Riikonen R. Infantile spasms: outcome in clinical studies. Pediatr Neurol, 2020, 108: 54-64.
29. 易招师, 查剑, 吴华平, 等. 婴儿痉挛症缓解后复发的危险因素. 中华实用儿科临床杂志, 2016, 31(12): 899-902.
30. 查剑, 曾兴颖, 易招师, 等. 大剂量泼尼松治疗婴儿痉挛症的优化方案. 癫痫杂志, 2017, 3(3): 193-198.
31. Kapoor D, Sharma S, Garg D, et al. Intravenous methylprednisolone versus oral prednisolone for west syndrome: a randomized open-label trial. Indian J Pediatr, 2021, 88(8): 778-784.
32. Deswal M, Lekhwani S, Vaswani ND, et al. Oral dexamethasone versus prednisolone for management of children with west syndrome: an open-labeled randomized controlled pilot trial. Ann Indian Acad Neurol, 2022, 25(5): 916-920.
33. Yi Z, Wu H, Yu X, et al. High-dose prednisone therapy for infantile spasms and late-onset epileptic spasms in China: The addition of topiramate provides no benefit. Seizure, 2019, 71: 174-178.
34. Rajaraman RR, Lay J, Alayari A, et al. Prevention of infantile spasms relapse: Zonisamide and topiramate provide no benefit. Epilepsia, 2016, 57(8): 1280-1287.
35. Willmore LJ, Abelson MB, Ben-Menachem E, et al. Vigabatrin: 2008 update. Epilepsia, 2009, 50(2): 163-173.
36. Yang J, Shen J. Elevated endogenous GABA concentration attenuates glutamate-glutamine cycling between neurons and astroglia. J Neural Transm (Vienna), 2009, 116(3): 291-300.
37. Griffith JL, Wong M. The mTOR pathway in treatment of epilepsy: a clinical update. Future Neurol, 2018, 13(2): 49-58.
38. Knupp KG, Coryell J, Singh RK, et al. Comparison of cosyntropin, vigabatrin, and combination therapy in new-onset infantile spasms in a prospective randomized trial. J Child Neurol, 2022, 37(3): 186-193.
39. Prezioso G, Chiarelli F, Matricardi S. Efficacy and safety of vigabatrin in patients with tuberous sclerosis complex and infantile epileptic spasm syndrome: a systematic review. Expert Rev Neurother, 2023, 23(7): 661-671.
40. Grinspan ZM, Knupp KG, Patel AD, et al. Comparative effectiveness of initial treatment for infantile spasms in a contemporary US cohort. Neurology, 2021, 97(12): e1217-e1228.
41. Al-Shehhi W, Chau V, Boyd J, et al. Treatment with high-dose prednisolone in vigabatrin-refractory infantile spasms. Can J Neurol Sci, 2022, 49(4): 532-539.
42. Biswas A, Yossofzai O, Vincent A, et al. Vigabatrin-related adverse events for the treatment of epileptic spasms: systematic review and meta-analysis. Expert Rev Neurother, 2020, 20(12): 1315-1324.
43. Westall CA, Wright T, Cortese F, et al. Vigabatrin retinal toxicity in children with infantile spasms: An observational cohort study. Neurology, 2014, 83(24): 2262-2268.
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