Purpose: West syndrome is a rare epileptic syndrome associated with infantile spasms, a specific abnormal electroencephalographic pattern (termed hypsarrhythmia), and mental retardation. Management of this disorder is difficult because current treatment regimens, including many anticonvulsants and hormones, are often ineffective. Topiramate (TPM) is a new antiepileptic drug that may be effective in pediatric epilepsies. We conducted a pilot study to test the effects of rapid TPM dosing in patients with refractory infantile spasms.
Methods: Eleven children with refractory infantile spasms were given an initial dose of 25 mg TPM per day in addition to their current therapy. Dosage was increased by 25 mg every 2-3 days until spasms were controlled, the maximal tolerated dose was reached, or the maximal dose of 24 mg/kg/day was achieved. Efficacy was primarily assessed by video EEG and secondarily by parental count of spasm frequency.
Results: Five (45%) subjects became spasm free during the study, with absence of infantile spasms and hypsarrhythmia (either classic or modified) proven by video EEG. Nine subjects, including the five spasm free, achieved a spasm reduction of > or =50%. Spasm frequency decreased from 25.6+/-19.3 to 6.9+/-5.9 spasms/day. Sixty-four percent of the subjects were able to achieve TPM monotherapy.
Conclusions: Results in this cohort of 11 patients with refractory disease show TPM to be a promising new agent for the treatment of infantile spasms.