Abstracts

Rationale: Infantile spasms are age-specific seizures commonly associated with an epileptic encephalopathy. There is limited evidence to guide treatment and the approach to infantile spasms varies among clinicians. The aim of this survey was to assess how clinicians currently evaluate and treat infantile spasms. Methods: To determine common practice among clinicians who treat infantile spasms, a survey was distributed via the Child Neurology Society to its members. Anonymous responses were collected over a two week period. Results: The response rate was 18.5% with 222 responses. Ninety-four percent of responders were from the United States. Seventy percent of responders reported seeing 10 or fewer new-onset cases of infantile spasms annually. For infantile spasms of unknown etiology, the most commonly used first-line treatments were adrenocorticotropic hormone (ACTH) (67%), oral corticosteroids (15%), and vigabatrin (9%). For spasms of structural/metabolic etiology, excluding tuberous sclerosis, the most commonly used first-line treatments were ACTH (44%), oral corticosteroids (23%), vigabatrin (14%), and topiramate (12%). Most responders (86%) use vigabatrin as the first-line treatment for infantile spasms due to tuberous sclerosis. Widely divergent doses of ACTH are used, with just over half of responders starting with high dose (150 U/m2/day) and others using variably lower doses. The preferred dosing of oral corticosteroid likewise varied significantly. We found that the diagnostic evaluation of infantile spasms also differed among clinicians. Conclusions: There is significant variation among clinicians in both first-line treatments as well as the initial evaluation of infantile spasms. While ACTH is still the most commonly used first-line treatment for infantile spasms not due to tuberous sclerosis, a wide range of dosing is used. Although a significant number of clinicians used corticosteroids first-line for infantile spasms, there is also a wide range of preferred dosing. Well-designed comparative effectiveness studies are needed to determine the optimal medication and dosing regimen in this devastating epileptic encephalopathy of infancy. However, collaboration within and between centers with agreement upon standardized protocols will be required to answer these important questions.