Demographic characteristics and clinical presentation of infants with infantile epileptic spasms syndrome and their response to therapy: Data from Sri Lanka Infantile Spasms Registry.

Objectives: Infantile epileptic spasm syndrome (IESS) is an epileptic encephalopathy with often devastating developmental consequences. Most children with IESS have a known etiology, although differing in proportion by geographical settings. Therefore, registries are useful to understand the characteristics of IESS in different countries. The Sri Lanka Infantile Spasms Registry (SLISR) was established to study the demographics and etiology of infants with IESS and their response to therapy in a resource-limited country. Methods: Five pediatric neurologists (out of nine) in different parts of the country prospectively recruited children with IESS. The etiology was evaluated using the services available in each setting. Response to treatment for standard (adrenal corticotropic hormone, prednisolone, or vigabatrin) versus nonstandard medications was evaluated at two and six weeks. Results: Included in the current analysis were 270 children who were registered since 2017. Median age at presentation was 5.36 months (SD 3.6). The mean interval between seizure onset and treatment onset was 1.7 months (SD 1.3). A sizable proportion of the children (61.2%) did not complete the evaluation of etiology. Structural brain abnormality was the most frequently identified etiology in those who were evaluated (38.8%); hypoxic-ischemic injury was the most common antecedent. The majority of the patients (86%) received a recommended standard therapy as the first treatment, with prednisolone being the most frequent choice. By treatment day 14, the first treatment had achieved spasm control in 63.8% and an electro-clinical response in 43.6%. While both standard therapies led to positive outcomes, oral prednisolone produced the best therapeutic response. Conclusion: We describe the etiologies, treatment choices, and response to first-line medications in a large group of children with IESS from a South Asian country. Although most patients received a recommended first-line therapy (most often prednisolone), a sizable number initially received nonstandard therapy. Our data illustrate the challenges in the management of IESS in a resource-limited environment.