|
Trials Evaluating Natural ACTH1-39 (RCI, Acthar® Gel)
|
| Baram et al, 1996[@41984] (US; single center) |
2 weeks;
4-24-hour video EEG for response.
|
Symptomatic or cryptogenic IS
No prior steroid or ACTH treatment.
Had hypsarrhythmia or its variants and epileptic myoclonic events.
|
N=29
2 to 21 mos
(mean=6.3 mos)
|
150 units/m2/day in 2 divided doses |
RCI was superior to oral corticosteroid treatment |
Cessation of spasms and resolution of hypsarrhythmia |
Hrachovy et al, 1983[@41986]
(US; single center)a
|
2 weeks;
24-hour video EEG for response.
|
IS and hypsarrhythmic EEG patterns.
No prior ACTH or corticosteroid therapy.
|
N=24
3.5 to 24 mos
(mean=8.2 mos)
|
20 units/day for 2 weeks and increased to 30 U/day |
A higher proportion of patients responded to RCI than oral corticosteroid treatment |
Cessation of spasms and resolution of hypsarrhythmia |
|
Trials evaluating synthetic ACTH1-24 (Tetracosactide, Synacthen®)
|
O'Callaghan et al, 2017[@41963]
(UK, Australia, Germany, New Zealand, Switzerland; multicenter)b
|
4 weeks;
No mention of EEG duration.
Absence of spasms for a 4-week period.
|
Clinical diagnosis of IS and hypsarrhythmic (or similar) EEG, ≤7 days before enrollment.
No previous treatment for IS, including hormonal treatments and vigabatrin.
|
N=377
2 to 14 mos
|
0.5 mg [40 IU] on alternate days for 2 weeks, increased to 0.75 mg [60 IU] on alternate days after 1 week |
A higher proportion of patients responded to tetracosactide than oral corticosteroid treatment |
Cessation of spasms |
| Lux et al, 2004[@41971] (UK; multicenter) |
2 weeks;
No mention of EEG duration.
Absence of spasms for a 48-hour period.
|
Clinical diagnosis of IS and a hypsarrhythmic (or similar) EEG with almost continuous, high-voltage multifocal spike and wave.
Both hypsarrhythmia and non-hypsarrhythmia patients included.
|
N=107
2 to 12 mos
|
0.5 mg [40 IU] on alternate days for 2 weeks, increased to 0.75 mg [60 IU] on alternate days after 1 week |
A higher proportion of patients responded to tetracosactide than oral corticosteroid treatment |
Cessation of spasms |
|
Trials Evaluating Synthetic ACTH1-39 (Corticotropin Carboxymethyl-cellulose, Acton Prolongatum®)
|
| Gowda et al, 2019[@41985] (India; single center) |
4 weeks;
No mention of EEG duration.
Absence of spasms for a 48-hour period.
|
Diagnosis of West Syndrome (based on West Adelphi Group).
No prior steroid use.
Children with symptomatic, idiopathic, and cryptogenic etiologies.
|
N=34
2 to 60 mos
(mean: 11.9 mos)
|
100 units per body surface area daily for 2 weeks |
A higher proportion of patients responded to CCMC than oral corticosteroid treatment |
Cessation of spasms |
Wanigasinghe et al, 2015[@41987]
(Sri Lanka; single center) |
2 weeks;
30-minute sleep EEG.
Absence of spasms for a 48-hour period.
|
Newly diagnosed IS occurring in clusters, confirmed based on direct observation or in video telemetry.
Only those with hypsarrhythmia included.
|
N=97
2 to 30 mos
(mean=9.1 mos)
|
40 to 60 IU/every other day |
CCMC was not superior to oral corticosteroid treatment |
Cessation of spasms and resolution of hypsarrhythmia |