Lamotrigine therapy in juvenile neuronal ceroid lipofuscinosis

Abstract

Purpose: To evaluate the effects of lamotrigine (LTG) therapy on epileptic seizures and general well-being in patients with juvenile neuronal ceroid lipofuscinosis (JNCL). Methods: LTG was initiated in 28 patients with JNCL. The mean age of the patients at the initiation of LTG was 13.7 years (range, 6.7-28.2 years). LTG was started at a dosage of 0.1-0.5 mg/kg/day and increased every 2 weeks until a maintenance dose of 1.25-15 mg/kg/day was reached. On the basis of the indication for LTG therapy, the patients could be divided into four groups. In the first group, LTG was initiated on an add- on basis; in the second group, LTG was started as the first anti-epileptic drug (AED) because of seizures, and in the third group, despite no preceding seizures, because of epileptiform activity in the whole-night polysomnography; in the fourth group, LTG replaced valproate (VPA), which was discontinued because of adverse side effects. The efficacy was assessed after 1 year on LTG. The mean follow-up time was 2.8 years (range, 1.3-5.8). Results: LTG had a favorable effect in 23 of 28 patients. A decrease in frequency of seizures of ≥50% was observed in 10 and a decrease in severity of seizures in nine of the 22 patients who had preceding seizures. Increases in well-being were found in 18 of 28. During the follow-up, LTG was continued as monotherapy in 13 of 19 patients. Conclusions: In light of our experiences, LTG seems to be a valuable drug in JNCL.