Abstracts

Rationale: Frontal lobe epilepsy(FLE) is a rare but challenging epilepsy syndrome . Carmabazepine and oxcarbazepine are considered the treatment of choice. About 30% cases of FLE prove to be resistant to medical treatment. We describe two children with refractory non-lesional FLE who became seizure free on rufinamide. Rufinamide, which was approved in 2008 by the FDA for treatment of Lennox Gastaut syndrome, is considered a broad spectrum anticonvulsant. However, its efficacy in frontal lobe epilepsy in children, has not been specifically reported Methods: Two children, one 7 years old and one 6 years old, were diagnosed with frontal lobe epilepsy based on seizure semiology, ictal and interictal EEG. In case # 1 seizure semiology consisted of two weeks of escalating nocturnal episodes of sudden arousal, looking scared, and agitated behavior lasting 20 - 40 seconds. At presentation she was having 10 -15 episodes at night, as well as several episodes while awake, during the day. The initial EEG showed occasional bi-frontal sharp-waves. Ictal onset was with high voltage, bi-frontal rhythmic slowing. In case # 2 semiology consisted of a scared look, hyperkinetic leg movements, head retraction, turn to one side, hypertonia with tremoring , lasting upto 2 min. Initial nocturnal episodes were soon followed by diurnal ones.. Interictal EEG showed synchronous bifrontal spike and slow wave discharges. Ictal EEG had a generalized decrement with fast activity over bilateral frontotemporal regions at the onset followed by evolution in either hemisphere with frontal more than temporal involvement.Both children had normal brain MRI. Results: In case#1, Initial treatment with oxcarbazepine , resulted in cessation of seizures within 4 days . Oxcarbazepine was stopped later due to rash . Seizures recurred on day two after stopping OXC. LEV was started. The rash resolved over the next two days but seizures escalated , occuring every 15 - 30 minutes. Topiramate was added with inadequate response. Valproic acid, phenytoin and pregabalin were used in succession with no effect. The child developed a rash again, this time temporally related to phenytoin. All anticonvulsants other than Topiramate were stopped and rufinamide was introduced.3 days after initiating treatment with rufinamide, the child became seizure free. In case 2- Oxcarbazepine controlled seizures but was stopped because of significant behavioral difficulty. Levatiracetam had no response. Rufinamide was started with complete cessation of seizures and improvement of behavior. Conclusions: We report 2 children with non-lesional FLE. Both responded to oxcarbazepine but a rash precluded its use in one and behavior difficulty in the other. Seizures were refractory to other anticonvulsants. Both became seizure free on Rufinamide. Although this is a report on just 2 patients , the results were dramatic. Rufinamide may be a viable treatment option for resistent FLE and could be considered early on in the treatment . This interesting observation needs to further validation.