Objective Clinical features and outcome of children with infantile spasms.

Study Design Interventional and observational study.

Place and Duration of Study The Department of Neurology, Children’s Hospital, Lahore, Pakistan, from January 2010 to December 2011.

Methodology Children aged <2 years presented with history of infantile spasms were assessed. Clinical presentation, EEG findings and response of anti-epileptic drugs was analyzed.

Results A total of 51,370 children visited Neurology outpatient department of Children Hospital, Lahore. Out of them, 450 infants had infantile spasms at their first presentation. Mean age at presentation was 6.6 + 2.5 months. Out of 450 children, 76% children presented at age < 6 month, 72% presented due to infantile spasms and 18% because of global developmental delay. Spasm types were mixed (38%), flexors (44%), extensor (16%) and asymmetric (2%). Symptomatic seizures were seen in 72% and cryptogenic in 28%. Hypsarrhythmia (67%) was the predominant EEG finding followed by modified hypsarrhythmia (24%) and other forms of epileptic discharges in 9% children. Majority of children were receiving oral Phenobarbitone, Carbamzaepine or Valproate sodium. We initiate the management with oral Prednisolone followed by Clonazepam or valproate acid. ACTH therapy was administered in only 5 children.

Conclusion Infantile spasms are one of the refractory epilepsy in children. Abnormal EEG findings predominantly the hypsarrhythmia or modified hypsarrhythmia are the hallmark. Majority of children received conventional AED with poor response. Oral prednisolone is proved to be the most effective AED. These children should be referred to the tertiary care paediatric neurology centers.