Abstracts

Nocturnal frontal lobe epilepsy (NFLE) is characterized by brief nocturnal motor seizures, which are described as simple arousals from sleep. They may include bizarre hyperkinetic events with tonic or dystonic features, and are sometimes misdiagnosed as night terrors. Carbamazepine (CBZ) has been reported to be effective in NFLE, but these seizures may also be refractory to various anti-epileptic drugs (AEDs). Here we report four children with NFLE, some of whom were refractory to other AEDs, who responded dramatically to treatment with oxcarbazepine (OXC). Four children between the ages of 4 and12 years old were identified at Children[apos]s Hospital Boston with brief stereotypical, nocturnal hyperkinetic episodes, often associated with grunting and choking sounds. Routine and prolonged continuous EEG, in addition to brain MRI, were performed in all patients. All four patients were initially started on AEDs other than OXC with minimal or no response in seizure control. Patients were initiated on OXC at 10mg/kg/day and the dose was increased to standard weight-based doses. Patients were followed for response of seizures to medication, side effects, and routine blood tests including blood count, chemistries, and serum 10-monohydroxide derivative (MHD) level. Three patients showed normal interictal EEG; two of these had ictal focal bifrontal beta activity and/or irregular bifrontal slowing. The fourth patient[apos]s EEG showed right frontocentral spike and slow wave complexes interictally; ictal EEG showed rhythmic 6-7 Hz theta and alpha activity in the right frontocentral area. Brain MRI studies showed no obvious focal brain lesion in all four patients. Ineffective AEDs in these patients included valproic acid, levetiracem, gabapentin, and phenytoin. OXC dramatically improved seizures within four days of initiation in three patients. The fourth patient required higher doses of OXC before achieving complete seizure control. The mean maintenance dose of OXC was 43.7 +/- 5.8 milligrams/kilogram/day, and the mean MHD level was 24.2 +/- 8.6 micrograms/mL (range 17-38). Patients have been followed for 6 to 12 months with no recurrence of seizures. There were minimal side effects in two patients (dizziness and somnolence in one patient, diplopia in another) which correlated with higher OXC doses and MHD levels. Sodium level (range138-142, mean 139.3 +/- 2.1 mmol/L), LFTs and CBC were normal. We conclude from our cases that OXC is efficacious for NFLE patients with nocturnal hyperkinetic motor seizures. The patients were able to tolerate high OXC doses without significant side effects, which is often difficult with CBZ. From our experience, we suggest that OXC may be recommended as the preferred first-line AED in NFLE.