Characterizing the phenotype of drug-resistant childhood epilepsy associated with leukemia: A case series

Children with leukemia are at risk for epilepsy due to primary disease or neurotoxic therapies. We describe the phenotypes of drug-resistant epilepsy in 10 children with history of leukemia. Of 10 cases, 6 had features of Lennox-Gastaut syndrome, and 4 had focal epilepsy. Mean age of epilepsy onset was 5 years in Lennox-Gastaut cases and 6.5 years in focal epilepsy cases. Mean latency between leukemia diagnosis and seizure onset was about 3 years. Brain MRI of 2 patients with epileptic encephalopathy had structural abnormalities – unclear if causative for epilepsy, and 4 had no overt structural abnormalities. In focal epilepsy group, 3 had temporal lobe epilepsy and one had fronto-temporal localization. All 10 patients had received intrathecal chemotherapy; 2 also had received whole brain irradiation. Seizures were poorly controlled in the epileptic encephalopathy group. Three underwent corpus callosotomy with variable response. Two patients with temporal lobe epilepsy had temporal lobectomy with Engel 1 outcome at 2 year follow-up in both. Two phenotypes of refractory epilepsy were observed in children with previous history of leukemia, focal epilepsy and epileptic encephalopathy. Children with temporal lobe epilepsy had good response to temporal lobectomy; response to palliative surgery was variable.