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Medical treatment of patients with infantile spasms
Mohamad A Mikati1, Garine A Lepejian, Gregory L Holmes
1Adult and Pediatric Epilepsy Program and Department of Pediatrics American University of Beirut, Medical Center Beirut, Lebanon.
Insights
Infantile spasms, a key feature of West syndrome, are treated with adrenocorticotropic hormone (ACTH) or vigabatrin. Other medications like valproic acid and nitrazepam are also discussed for infantile spasms management.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Pharmacology
Background:
- West syndrome is an age-related epilepsy characterized by infantile spasms, affecting 1 in 2,000-4,000 infants.
- Infantile spasms represent a significant challenge in pediatric neurology, necessitating effective therapeutic strategies.
Purpose of the Study:
- To provide a comprehensive literature review on infantile spasms and their therapeutic options.
- To evaluate the efficacy and limitations of various treatments for infantile spasms.
Main Methods:
- Literature review of studies on infantile spasms and their treatments.
- Analysis of drug efficacy, including adrenocorticotropic hormone (ACTH), vigabatrin, valproic acid, and nitrazepam.
Main Results:
- Adrenocorticotropic hormone (ACTH) is the preferred treatment for cryptogenic infantile spasms, showing better efficacy than corticosteroids and impacting long-term prognosis.
- Vigabatrin is recommended for infantile spasms secondary to tuberous sclerosis, but retinopathy concerns limit its use.
- Valproic acid is effective in 40%-70% of patients unresponsive to ACTH; nitrazepam offers acute control but lacks long-term prognosis data.
Conclusions:
- Treatment selection for infantile spasms depends on the etiology, with ACTH and vigabatrin as primary options.
- Emerging concerns about vigabatrin's side effects necessitate careful consideration.
- Further research is needed to fully understand the long-term prognostic impact of various infantile spasms therapies.
Abstract:
Infantile spasms are the main feature in West syndrome, an age-related epilepsy syndrome that affects 1 in every 2,000-4,000 infants. The authors provide a comprehensive review of the literature about infantile spasms and their therapy. In the United States, the drug of choice for infantile spasms, at least the cryptogenic cases, has been adrenocorticotropic hormone (ACTH). It is generally considered to be more effective than corticosteroids. Adrenocorticotropic hormone appears to alter long-term prognosis of cryptogenic infantile spasms, and helps in some cases of symptomatic infantile spasm. Vigabatrin has been considered the drug of choice for infantile spasms secondary to tuberous sclerosis, and possibly, according to many neurologists, for all cases of infantile spasm. Recent concerns regarding retinopathy associated with vigabatrin therapy are, however, limiting the use of this drug. Valproic acid benefits 40%-70% of patients who failed a trial of ACTH. Nitrazepam is as effective as ACTH in acutely controlling infantile spasms; however, its long-term effects on prognosis have not been studied. Pyridoxine, lamotrigine, topiramate, zonisamide, ketogenic diet, immunoglobulin therapy, felbamate, and thyrotropin-releasing hormone have all been used for the treatment of infantile spasms, but are usually reserved for cases refractory to vigabatrin and/or ACTH.