Abstracts

RATIONALE: To evaluate the impact of hemispherectomy on seizure and developmental outcome in infants with hemimegalencephaly and Ohtahara syndrome. METHODS: We retrospectively examined the cases of two infants with hemimegalencephaly, seizures and suppression-burst EEG patterns who underwent subsequent hemispherectomy. Results of presurgical evaluation and post-operative follow-up were reviewed. RESULTS: Patient 1 presented with seizures within the first few hours of life and developed nearly continuous electrographic seizures. Left hemispherectomy was performed at 28 days of age. Patient 2 presented with flexor spasms at 6 months of age. Seizures were intractable to medications over the next 12 months during which the child stopped smiling, cooing, rolling over and sitting up and developed a right hemiparesis. Left hemispherectomy was performed at 18 months of age. Pathology in both cases revealed high-grade cortical dysplasia with numerous dysplastic giant neurons. Both patients have been seizure free since surgery. At 5 years of age, patient 1 is currently ambulatory with a 10-20 word vocabulary. Patient 2, now 25 months of age, has regained all previously lost milestones and has begun to babble. CONCLUSIONS: Seizure and developmental outcome of infants with Ohtahara syndrome associated with hemimegalencephaly may be improved by early hemispherectomy.