The efficacy and side effects of various
antiepileptic drugs (AEDs), especially
pyridoxine,
ACTH and
valproate sodium (VPA), in the treatment of
West syndrome were reviewed.
ACTH remains to be the most effective treatment for
West syndrome. However, there are significant adversive effects with prolonged
ACTH therapy. The efficacy of
pyridoxine at the dose of 40-50 mg/kg/day is less encouraging, but there are no serious adversive effects, as opposed to
ACTH. Some reports have indicated the possible efficacy of VPA in regular, large (40-100 mg/kg/day) and very high (100-300 mg/kg/day) doses. However, in patients under the age of 2 years, monotherapy using VPA in lower doses should be employed to minimize the risk of VPA-induced adversive effects, such as fatal hepatic toxicity. Consequently, a safer and more effective treatment is required. We have reported a pilot study on combination
therapy with high-dose
pyridoxine phosphate (40-50 mg/kg/day) and low-dose
ACTH (0.01 mg/kg/day) in 25 children with
West syndrome, mainly on the basis of neurochemical analysis of the ictal epileptic
spasms. The response rate was similar to those achieved with high-dose
ACTH, but a quicker cessation of
spasms was obtained with the combination
therapy than
ACTH monotherapy. Transient increases in liver
enzymes occurred in 50%, but none of the patients developed more serious side effects. Further study is required to determine the efficacy of this combination
therapy.