Introduction: West syndrome is a common age-related epileptic encephalopathy with onset usually in the first year of life. This study aimed to evaluate the clinico-etiological profile and short term seizure outcomes in children  with epileptic spasms with onset after 12 months of age.

Methods: The medical records of children diagnosed with West syndrome with onset of spasms after 12 months of age at a tertiary care, referral hospital in North India from January 2011 to June 2013, were retrospectively reviewed. The clinic-etiological profile and treatment response were studied.

Results: During the study period, 253 cases were diagnosed with West syndrome. 24 (9.5%) children had onset of spasms after 12 months of age. The mean age at presentation and onset of spasms were 20.4 months (SD-5.4) and 15.7 months (SD-4.8), respectively. 91.7% were males. Perinatal asphyxia was the most common etiology (16;66.7%) followed by cryptogenic (3;12.5%), tubercular meningitis (2;8.3%), neonatal hypoglycemia sequalae (1;4.2%), tuberous sclerosis (1;4.2%) and bacterial meningitis (1;4.2%). EEG showed hysparrhythmia (11;45.8%) or modified hypsarrhythmia (13;54.2%). The median duration of follow-up was 6 months (6-10). The response to oral prednisolone was: 100% (11;45.8%), >50-99% (3;12.5%) and < 50% (7;29.17). At last follow up, 11 (45.8%) children were spasm-free, 7 (29.2%) had persisting spasms and 6 (26.1%) had other seizure types (2 evolved to Lennox-Gastaut syndrome).

Conclusions: Epileptic spasms may appear after 12 month of age. Perinatal asphyxia remains the most common etiology. The response to oral steroids appears to be good.