Infantile Epileptic Spasms Syndrome: Clinical profile and outcomes at a Tertiary Hospital in Kenya

Abstract

Background: Infantile epileptic spasms syndrome (IESS) is characterised by onset of epileptic spasms between 3 and 12 months, with or without typical hypsarrhythmia, and developmental stagnation or regression. There are limited data on the clinical profile and treatment outcomes of IESS in sub-Saharan Africa. Objective: To describe the clinical profile and treatment outcomes of infants diagnosed with and managed for IESS at Aga Khan University Hospital, Nairobi. Methods: Records of children aged 3–24 months diagnosed with IESS between January 2012 and December 2021 were identified from the hospital information system using the terms “infantile spasm”, “epileptic spasms”, and “West syndrome”. Medical history, investigations, treatment, and outcomes were extracted using a structured tool. Outcomes at six weeks were categorised as complete response (spasm-free), partial response (any reduction in spasms), or no response. Results: Forty-two children with IESS were identified. The median age at onset was 5 months (IQR 3.5–8.5). The male-to-female ratio was 1:2.8. Age at diagnosis and treatment initiation was 6.5 months (IQR 4.0–10.5). Flexor spasms were most frequent (24/42; 57.1%). The commonest aetiology was structural (26/42; 61.9%), with unknown causes in 14/42 (33.3%). Most patients had received non–first-line anti-seizure medicines before referral. At the study site, patients received vigabatrin plus prednisolone irrespective of aetiology. At six weeks, 24/42 (57.1%) were spasm-free, 17/42 (40.5%) had a partial response, and 1/42 (2.4%) was lost to follow-up. By two years of age, 3/37 (8.1%) assessed children had normal development, while 34/37 (91.9%) had delays in multiple domains. Ongoing seizures were reported in 32/42 (76%) patients, with a mean duration of 7.04 weeks. Conclusion: Despite more than half of patients achieving spasm cessation at six weeks, few children attained typical developmental milestones at follow-up, likely reflecting delays to diagnosis and treatment. Standardised referral pathways are urgently needed to enable timely management of IESS and improve outcomes. A prospective, multicentre study in the region is warranted.