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Treatment of infantile spasms: an evidence-based approach
Mark Mackay1, Shelly Weiss, O Carter Snead
1Division of Neurology, Research Program in Brain and Behavior, Hospital for Sick Children, Department of Pediatrics and Medicine (Neurology), Bloorview Epilepsy Research Program, Faculty of Medicine, University of Toronto, Ontario, Canada M5G 1X8.
Insights
Vigabatrin and ACTH are the most effective treatments for infantile spasms. However, evidence does not strongly link successful treatment to improved long-term cognitive outcomes or reduced epilepsy incidence.
Area of Science:
- Pediatric Neurology
- Evidence-Based Medicine
Background:
- Infantile spasms (IS) are a severe epilepsy syndrome in infants.
- Understanding the natural history and optimal treatment of IS is crucial for pediatric care.
Purpose of the Study:
- To conduct an evidence-based analysis of established medical treatments for infantile spasms.
- To determine the current best practice for treating infantile spasms in children.
Main Methods:
- Critical review of pre-steroid era studies to define IS natural history.
- Rigorous assessment of treatment trials since 1958 using MEDLINE and hand searches.
- Classification of evidence (Class I, II, III) and application of American Academy of Neurology practice parameter framework.
Main Results:
- Vigabatrin is recommended for IS in children with tuberous sclerosis (Class I & III evidence).
- ACTH or vigabatrin are recommended for IS in non-tuberous sclerosis patients (Class I & III evidence).
- Limited evidence supports oral corticosteroids; no recommendations for pyridoxine, benzodiazepines, or newer antiepileptics.
Conclusions:
- ACTH and vigabatrin are the most effective IS treatments, but risk/benefit profiles require consideration.
- Current evidence does not strongly support improved long-term cognitive outcomes or reduced later epilepsy incidence with IS treatment.
- Best practice recommendations prioritize vigabatrin for tuberous sclerosis and ACTH/vigabatrin for others.
Abstract:
The object of this work was to subject established empirical medical treatment regimens for infantile spasms to evidence-based medicine analysis in order to determine the current best practice for the treatment of infantile spasms in children. Clinical studies of infantile spasms reported during the presteroid era were reviewed critically to define the natural history of the disorder. Treatment trials of infantile spasms conducted since 1958 were rigorously assessed using MEDLINE and hand searches of the English language literature. Inclusion criteria were the documented presence of infantile spasms and hypsarrhythmia. Outcome measures included complete cessation of spasms, resolution of hypsarrhythmia, relapse rate, developmental outcome, the presence or absence of epilepsy, and/or an epileptiform electroencephalogram. Evidence was defined as class I, II, or III, and practice parameter recommendations were made using the framework devised by the American Academy of Neurology. Class I and III evidence support a standard of practice recommendation for the use of vigabatrin in the treatment of infantile spasms in children with tuberous sclerosis. Class I and III evidence support a guidelines recommendation for the use of either ACTH or vigabatrin in infantile spasms in nontuberous sclerosis patients. There is no strong evidence that successful treatment of infantile spasms improves the long-term prognosis for cognitive outcome or decreases the incidence of later epilepsy. A practice option recommendation for the use of oral corticosteroids in the treatment of infantile spasms is supported by limited and inconclusive class I and III data. Based on the evidence, no recommendation can be made for the use of pyridoxine, benzodiazepines, or the newer antiepileptic drugs in the treatment of infantile spasms. ACTH and vigabatrin are the most effective agents in the treatment of infantile spasms, but concerns remain about the risk/benefit profiles of these drugs.