Epilepsy is a common and potentially serious and disabling disease. Congenital malformations of the brain are often associated with severe presentations and difficult drug control. Our objective is to report a case of a child with hemimegalencephaly, the clinical and electroencephalographic presentation before and after neurosurgery.

RAR, a 2-year-old girl with difficult-to-control epilepsy using a combination of high doses of anticonvulsants, presented daily crisis from the neonatal period. She was monitored with video-EEG before and after brain surgery. Clinical presentation and electroencephalographic changes helped to indicate the procedure.

The video-EEG showed crises characterized by behavioral arrest, eyes up and stillness. Inter-ictal EEG had continuous poly-spykes in the right hemisphere and focal EEG accentuates epileptiform activity over the right quadrant region. Magnetic resonance imaging of the requested brain with right hemimegalencephaly. The patient underwent right hemispherectomy in December 2017, provided no recurrence of crisis. Her behavior and development is also better. She will be admitted shortly for a new video-EEG monitoring.

Hemimegalencephaly is a malformation of cortical development characterized by excessive hamartomatous growth of all or part of a cerebral hemisphere and frequently associated with refractory epilepsy. Patients may present with early encephalopathy, delayed neuropsychomotor development, motor deficits and learning disabilities. Anatomic or functional hemispherectomy is indicated if epilepsy is clinically intractable.