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[Treatment of infantile spasms with long-term low dose ACTH]
M Kuriyama1, Y Konishi, Y Fujii
1Department of Pediatrics, Fukui Medical School.
Insights
Long-term, low-dose adrenocorticotropic hormone (ACTH) therapy effectively controlled infantile spasms in most patients, with a higher total dose linked to better outcomes. Side effects like hypertension and hypokalemia were manageable.
Area of Science:
- Pediatric Neurology
- Endocrinology
Context:
- Infantile spasms are a severe form of epilepsy in infants.
- Adrenocorticotropic hormone (ACTH) is a treatment option, but optimal dosing and long-term effects require further investigation.
Purpose:
- To evaluate the efficacy and safety of long-term, low-dose ACTH therapy for infantile spasms.
- To assess the relationship between total ACTH dose and treatment outcomes.
Summary:
- This study treated 13 patients with infantile spasms using low-dose ACTH (mean: 0.0081 mg/kg/day) for 30 days, followed by tapering.
- Complete seizure cessation was achieved in 13 of 15 treatment trials, with EEG showing a good response.
- Common side effects included hypertension, hypokalemia, and emotional outbursts, which were generally manageable and resolved with dose tapering. Mild brain shrinkage was observed on CT scans.
Impact:
- Long-term, low-dose ACTH therapy demonstrates significant efficacy in achieving seizure control for infantile spasms.
- Higher total ACTH doses were associated with better treatment outcomes.
- Understanding side effect profiles and dose-response relationships can optimize infantile spasms management.
Abstract:
We investigated the effect of long-term, low-dose ACTH in 13 patients (10 boys and 3 girls) with infantile spasms who were treated with low-dose ACTH (mean: 0.0081 mg/kg/day). Two patients (one boy and one girl) received this therapy twice because of relapse of tonic spasms. ACTH was injected intramuscularly every morning for 30 days, after which dosage was tapered. The mean observation period was 53.9 months. Complete cessation of seizures was attained in 13 of 15 treatment trials. In one trial, complete cessation was not attained but the number of attacks decreased to less than one-third of that before treatment. In only one trial was treatment not effective. EEG showed good response to this treatment. The side-effects of this therapy were hypertension in 6 patients, hypokalemia in 7, and emotional outburst in 7. Emotional outburst appeared during the early phase of therapy, while the other two side-effects appeared in the later phase and disappeared when ACTH-tapering was begun. Brain shrinkage observed on CT scan was mild in all trials. Five patients have had no relapse. The total dose of ACTH was significantly larger in the group with good outcome than in the group with poor outcome.