Infantile epileptic spasms syndrome carries high morbidity and mortality compared with other childhood epilepsy syndromes. High-dose prednisolone is considered the first-line option by many neurologists, with efficacy comparable to adrenocorticotropic hormone (ACTH). The second-line therapy is debated if prednisolone fails to induce remission. The objective of this study was to evaluate the efficacy of adrenocorticotropic hormone versus vigabatrin as second-line antiseizure medication in patients with infantile epileptic spasms syndrome after high-dose prednisolone failure. Thirty-eight patients met the inclusion criteria. Seventeen patients (45%) took vigabatrin and 21 (55%) took adrenocorticotropic hormone. There were no significant differences regarding age of infantile spasms onset (P = .37) or sex between both groups (P = .32). Complete control of epileptic spasms was seen in 5 of 17 patients (29.5%) taking vigabatrin and in 4 of 21 (19%) taking adrenocorticotropic hormone (P = .70). Vigabatrin showed higher efficacy; however, it did not reach statistical significance, possibly because of our sample size. These results may help guide family counseling after the failure of high-dose prednisolone as first-line therapy.
SorelLDusaucy-BauloyeA. A propos de 21 cas d'hypsarhythmia de Gibbs; son traitement spectaculaire par l'ACTH. Acta Neurol Psychiatr Belg. 1958;58:130‐141.
2.
MontenegroMAEckKJacobS,et al.Long-term outcome of symptomatic infantile spasms established by video-electroencephalography (EEG) monitoring. J Child Neurol. 2008;23:1288‐1292.
3.
ChironCDulacOLunaD, et al.Vigabatrin in infantile spasms. Lancet. 1990;335:363‐364.
4.
ChironCDulacOBeaumontDPalaciosLPajotNMumfordJ. Therapeutic trial of vigabatrin in refractory infantile spasms. J Child Neurol. 1991;6(2):S52‐S59.
5.
KnuppKGCoryellJSinghRK, 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. doi:https://doi.org/10.1177/08830738211073400
6.
LuxALEdwardsSWHancockE, et al.The United Kingdom Infantile Spasms Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomised controlled trial. Lancet. 2004;364:1773‐1778.
7.
LuxALEdwardsSWHancockE, et al.The United Kingdom Infantile Spasms Study (UKISS) comparing hormone treatment with vigabatrin on developmental and epilepsy outcomes to age 14 months: a multicentre randomised trial. Lancet Neurol. 2005;4:712‐717.
8.
O'CallaghanFJKEdwardsSWAlberFD, et al.Vigabatrin with hormonal treatment versus hormonal treatment alone (ICISS) for infantile spasms: 18-month outcomes of an open-label, randomised controlled trial. Lancet Child Adolesc Health. 2018;2:715‐725.
9.
JonesKSneadOC3rdBoydJGoC. Adrenocorticotropic hormone versus prednisolone in the treatment of infantile spasms post vigabatrin failure. J Child Neurol. 2015;30:595‐600.
10.
WanigasingheJArambepolaCSri RanganathanSSumanasenaSAttanapolaG. Randomized, single-blind, parallel clinical trial on efficacy of oral prednisolone versus intramuscular corticotropin on immediate and continued spasm control in west syndrome. Pediatr Neurol. 2015;53:193‐199.
11.
WestWJ. On a peculiar form of infantile convulsions. Lancet. 1841;i:724‐725.
12.
OsborneJPEdwardsSWAlberFD, 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.
13.
XuZGongPJiaoX,et al.Efficacy of vigabatrin in the treatment of infantile epileptic spasms syndrome: a systematic review and meta-analysis. Epilepsia Open. 2023;8: 268‐277.
14.
O'CallaghanFJEdwardsSWAlberFD, 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;16:33‐42.
15.
HussainSAShinnarSKwongG,et al.Treatment of infantile spasms with very high dose prednisolone before high dose adrenocorticotropic hormone. Epilepsia. 2014;55: 103‐107.
16.
EliyanYHeeschJAlayariARajaramanRRSankarRHussainSA. Very-high-dose prednisolone before ACTH for treatment of infantile spasms: evaluation of a standardized protocol. Pediatr Neurol. 2019;99:16‐22.
17.
DzauWChengSSnellP,et al.Response to sequential treatment with prednisolone and vigabatrin in infantile spasms. J Paediatr Child Health. 2022;58:2197‐2202.
18.
ZuberiSMWirrellEYozawitzE, et al.ILAE Classification and definition of epilepsy syndromes with onset in neonates and infants: position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022;63:1349‐1397.
19.
KwanPArzimanoglouABergAT, et al.Definition of drug resistant epilepsy: consensus proposal by the ad hoc Task Force of the ILAE Commission on Therapeutic Strategies. Epilepsia. 2010;51:1069‐1077.
20.
LiSZhongXHongSLiTJiangL. Prednisolone/prednisone as adrenocorticotropic hormone alternative for infantile spasms: a meta-analysis of randomized controlled trials. Dev Med Child Neurol. 2020;62:575‐580.
21.
GrinspanZMMytingerJRBaumerFM, et al.Management of infantile spasms during the COVID-19 pandemic. J Child Neurol.2020;35:828‐834.
22.
KnuppKGLeisterECoryellJ, et al.Response to second treatment after initial failed treatment in a multicenter prospective infantile spasms cohort. Epilepsia. 2016;57:1834‐1842.
23.
MahmoudAARizkTMMansyAAAliJAAl-TannirMA. Ineffectiveness of topiramate and levetiracetam in infantile spasms non-responsive to steroids: open labeled randomized prospective study. Neurosciences (Riyadh). 2013;18:143‐146.
24.
HussainSANavarroMHeeschJ, et al.Limited efficacy of zonisamide in the treatment of refractory infantile spasms. Epilepsia Open. 2020;5:121‐126.
25.
WeberAColeJWMytingerJR. Infantile spasms respond poorly to topiramate. Pediatr Neurol. 2015;53:130‐134.
26.
FoxJRGuido-EstradaNWilliamsKJarrarR. Outcomes among patients with infantile spasms treated with hormonal therapy and adjuvant topiramate versus hormonal therapy alone. Epileptic Disord. 2020;22:33‐38.
27.
YiZWuHYuX, 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.
28.
Doré-BrabantGLaflammeGMilletteMOstermanBChrestianN. Adrenal insufficiency among children treated with hormonal therapy for infantile spasms. Epilepsia. 2022;63:2350‐2358.
29.
MaguireMJHemmingKWildJMHuttonJLMarsonAG. Prevalence of visual field loss following exposure to vigabatrin therapy: a systematic review. Epilepsia. 2010;51:2423‐2431.
30.
WildJMMartinezCReinshagenGHardingGFA. Characteristics of a unique visual field defect attributed to vigabatrin. Epilepsia. 1999;40:1784‐1794.
31.
KuchenbuchMLo BarcoTChemalyNChironCNabboutR. Fifteen years of real-world data on the use of vigabatrin in individuals with infantile epileptic spasms syndrome. Epilepsia. 2024;65:430‐444.
