Sulthiame add-on therapy in children with Lennox-Gastaut syndrome: A study of 44 patients.

Purpose: The aim of this study was to evaluate efficacy and tolerability of sulthiame as an add-on treatment in 44 patients with Lennox-Gastaut syndrome (LGS) refractory to other antiepileptic drugs and/or non-pharmacological treatment.

Methods: Patients were selected according to the following criteria: (1) age 4 years or older, (2) a diagnosis of LGS refractory to at least four previous antiepileptic drugs, alone or in combination. Neurologic examinations, brain magnetic resonance imaging, and repeated prolonged electroencephalography (EEG) or video-EEG studies were performed in all cases. Data on school achievements and/or neuropsychological evaluations were obtained during the follow-up of 1-3 years. Sulthiame was added in doses ranging from 5 to 30 mg/kg/day.

Results: Twenty-seven of 44 patients (61%) who received sulthiame as add-on therapy had a greater than 50% seizure decrease after a mean follow-up period of 20 months. Complete seizure freedom was achieved in one patient (2%). Four patients (9%) had a 25-50% seizure decrease, while seizure frequency remained unchanged in 12 (25%), and was increased in one (2%). Hyperpnoea and dyspnoea were observed in four patients, and nausea, drowsiness, and headache were seen in one patient each; however, these manifestations were transient and discontinuation of sulthiame was not necessary. Two other patients had decreased appetite, skin rash, and irritability. The adverse effects were mild and transient in these nine cases.

Conclusion: Sulthiame as an adjunctive therapy achieved a more than 50% seizure reduction in 27 of 44 patients with LGS with only mild or moderate adverse effects.