Abstracts

Rationale: Infantile epileptic spasms syndrome (IESS) is a rare epilepsy syndrome with first-line treatment, including vigabatrin and hormone-based treatments (systemic corticosteroids: adrenocorticotropic hormone [ACTH], or prednisolone). Systemic corticosteroids can be associated with a wide range of potential side effects, including adrenal suppression, driven by inadequate production of cortisol in the hypothalamic-pituitary-adrenal axis. This may be associated with additional complications such as vomiting, fever, seizures, coma, and death. These side effects are reported more frequently with steroids given in high doses and for prolonged periods. The rate of adrenal suppression remains unknown, especially in the pediatric population. We aimed to better characterize the prevalence of patients presenting with symptoms consistent with adrenal suppression/crisis after treatment with systemic corticosteroids for IESS.


Methods: This observational study included a medical chart review of 2 to 24-month-old individuals diagnosed with IESS between January 2018 and June 2023 at Boston Children's Hospital. Included participants had received at least one course of steroids (prednisolone or ACTH). Records were reviewed for symptoms suggestive of adrenal insufficiency also included emergency department (ED) visits due to symptoms of adrenal suppression/crisis that had occurred in the year following steroid treatment. Data collection is in process. We have included our preliminary data.


Results: Fifteen individuals were included, all of whom had at least undergone one course of steroids (prednisolone or ACTH) with a mean age at first steroid treatment of 7 (3-23) months. Three patients had a second course of steroids. All steroid courses were 30 days or less. Three patients had at least one visit to the ED after the completion of the steroids course for asymptomatic bradycardia, viral respiratory infection, metabolic acidosis in the setting of initiation of ketogenic diet and recurrence of epileptic spasms, but none of them had symptoms consistent with adrenal suppression or crisis.


Conclusions: Our results suggest adrenal suppression is a rare complication with the shorter steroid course given for IESS. Despite the rarity, it is serious, potentially life threatening and healthcare providers should still have a high index suspicion level for looking out for symptoms for adrenal suppression/crisis in the setting of IESS steroid treatment.


Funding: None