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Treatment of infantile spasms
E Hancock1, J Osborne, P Milner
1Clinical Science Centre for Research & Education, Lower Lane, Liverpool, Merseyside, UK, L9 7LJ.
Insights
Current treatments for infantile spasms (West's Syndrome) lack proven long-term efficacy and developmental benefits. More research is needed to determine the best therapies for this condition.
Area of Science:
- Neurology
- Pediatrics
- Clinical Pharmacology
Background:
- Infantile spasms (West's Syndrome) are a severe epilepsy syndrome characterized by specific seizures, hypsarrhythmia, and often psychomotor retardation.
- The underlying causes and pathophysiology of infantile spasms remain poorly understood, complicating treatment strategies.
Purpose of the Study:
- To compare the efficacy of single drugs used for infantile spasms.
- To evaluate effects on long-term psychomotor development, subsequent epilepsy, spasm control, and adverse events.
Main Methods:
- Systematic review of randomized controlled trials (RCTs) identified through major medical databases (MEDLINE, EMBASE) and other sources.
- Data extraction by independent reviewers focusing on spasm cessation, duration, seizure recurrence, and adverse effects.
Main Results:
- Eleven RCTs with 514 participants tested eight drugs; overall study methodology was poor.
- Vigabatrin showed potential efficacy in tuberous sclerosis patients, and a trend versus placebo.
- ACTH was more effective than low-dose prednisone; high-dose vigabatrin may improve spasm control.
Conclusions:
- No single treatment is definitively proven more efficacious than others for infantile spasms, except possibly vigabatrin in tuberous sclerosis.
- Few studies assessed long-term outcomes like psychomotor development or subsequent seizures.
- Further large-scale trials with extended follow-up are essential to guide treatment decisions.
Background:
Infantile spasms (West's Syndrome) is a syndrome which includes a peculiar type of epileptic seizure, usually hypsarrhythmia and in the majority of people, psychomotor retardation. It remains poorly understood and despite modern imaging techniques an underlying cause is often not found. Little is known about their pathophysiological basis and treatment remains problematic.
Objectives:
To compare the effects of single drugs used to treat infantile spasms in terms of long-term psychomotor development, subsequent epilepsy, control of the spasms and adverse effects.
Search Strategy:
Our search included the Cochrane Epilepsy Group trials register, MEDLINE (1966 to 2003) and EMBASE (1981 to 2003), contacting pharmaceutical companies and appeals at international conferences.
Selection Criteria:
All randomised controlled trials (RCTs) of the administration of drugs to people with infantile spasms.
Data Collection And Analysis:
Three reviewers independently selected trials for inclusion and extracted data. Outcomes included cessation of spasms, time to cessation of spasms, participants with cessation of spasms remaining spasm free, reduction in spasms, resolution of hypsarrhythmia, subsequent epilepsy rates and adverse effects.
Main Results:
Eleven RCTs were included, who in total recruited just 514 participants and tested eight different drugs. Overall, methodology of the studies was poor. No study assessed long-term psychomotor development or onset of other seizure types. One small study found vigabatrin to be more efficacious than hydrocortisone in stopping infantile spasms in a group of people with tuberous sclerosis. One underpowered study showed a trend for vigabatrin to be more efficacious than placebo in stopping infantile spasms. Two small studies when combined showed ACTH to be more efficacious than low-dose prednisone (2 mg/kg). One study also suggested that control of spasms occurred more frequently with high dose vigabatrin as compared to low dose vigabatrin. It was not possible to compare reduction in the number of spasms between the different treatments because of differences in methods of analysis. Overall, only 18 individuals were reported to have been withdrawn from the trial treatments due to adverse effects and 4 deaths were reported.
Reviewer'S Conclusions:
We found no single treatment to be proven to be more efficacious in treating infantile spasms than any of the others (other than vigabatrin in the treatment of infantile spasms in tuberous sclerosis in one underpowered study). Few studies considered psychomotor development or subsequent seizure rates as outcomes and none had long-term follow-up. Further trials with larger numbers of participants, and longer follow-up are required.