Nocturnal frontal lobe epilepsy is an epilepsy syndrome of children, and is characterized by brief nocturnal motor seizures, or sudden arousals from sleep. The syndrome may include bizarre hyperkinetic events with tonic or dystonic features and are often misdiagnosed as night terrors.

"It is very difficult to diagnose nocturnal frontal lobe epilepsy because EEGs [electroencephalograms] are often normal more than 50% of the time during the daytime," said principal investigator G. Praveen Raju, MD, Neurologist, Children's Hospital, Harvard Medical School, Boston, Massachusetts, United States.

Dr. Raju and colleagues studied seven children between 4 and 13 years old who suffered from nocturnal hyperkinetic episodes that were often associated with grunting and choking sounds. The researchers monitored children using prolonged, continuous EEGs. Brain magnetic resonance imaging (MRIs) was also performed to confirm the diagnosis.

Three of the children showed normal interictal EEGs, two with ictal focal bifrontal beta activity and/or irregular bifrontal slowing. One patient's EEG showed right frontocentral spike and slow wave complexes interictally and the ictal EEG showed rhythmic 6-7 Hz theta and alpha activity in the right frontocentral area. The MRI studies, however, showed no obvious focal brain lesions in these patients, Dr. Raju said.

After the diagnosis of NFLE was made, the children, whose ages ranged from 4 to 13, were started on antiepileptic drugs other than OXC (phenytoin, valproic acid, gabapentin, and levetiracetam) but had minimal or no response to the medication. They were then initiated on oxcarbazepine at 10 mg/kg/day, and the dose was increased to standard weight-based doses.

Within 4 days, 6 of the 7 patients responded to oxcarbazepine. The seventh patient eventually responded when the dose was increased to 30 mg, Dr. Raju said. The mean maintenance dose was 43.7 +/- 5.8 mg/kg/day, and the mean serum 10-monohydroxide derivative level was 24,2 +/- 8.6 mcg/mL.

"Their response was really amazing. These kids have been seizure free for 6 to 18 months," Dr. Raju said.

Adverse effects were minimal and included dizziness and somnolence in one patient, which was related to the higher dose, and diplopia in another patient, he added.

"The patients were able to tolerate high doses of oxcarbazepine without significant side effects," he added. "This is often difficult with carbamazepine. So, from our experience, we suggest that oxcarbazepine may be recommended as the preferred first-line antiepileptic drug in children with NFLE."


[Presentation title: Oxcarbazepine in Nocturnal Frontal Lobe Epilepsy. Abstract 2.206]

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