Sulthiame in the primary therapy of West syndrome: a randomized double-blind placebo-controlled add-on trial on baseline pyridoxine medication

Epilepsia. 2004 Feb;45(2):103-8. doi: 10.1111/j.0013-9580.2004.19003.x.


Purpose: West syndrome (WS) is still one of the most difficult to treat epilepsies in infancy. Sulthiame (STM), which is commonly used in some countries in the treatment of benign focal epilepsies in childhood, has been suggested to be effective in WS too. This prospective, randomized placebo-controlled study was designed to prove or refute this hypothesis.

Methods: Thirty-seven infants aged between 3.5 and 15 months with newly diagnosed WS received baseline therapy with pyridoxine (PDX). The children were randomized in a double-blind fashion to STM (n = 20) or placebo (n = 17), starting at day 4 at a moderate dose of 5 mg/kg body weight. Without complete cessation of infantile spasms (ISs) and resolution of hypsarrhythmia as the definition criteria of a response, the dose was doubled at day 7. The final examination was undertaken at the end of day 9.

Results: Based on the intention to treat, six (30%) of 20 patients responded to STM (p < 0.025), as did six (35%) of 17 infants fulfilling the study criteria (p < 0.01). Patients with tuberous sclerosis did not respond to STM (n = 3) No patient responded to placebo. One patient in the verum group was withdrawn because of reversible somnolence.

Conclusions: As no child in the placebo group responded to the baseline PDX therapy, nor did any child in the STM group during the first 3 days of baseline therapy, PDX does not seem to be effective in WS. STM has a positive effect in the primary therapy of WS, comparable to that of vigabatrin.

Publication types

  • Clinical Trial
  • Randomized Controlled Trial

MeSH terms

  • Anticonvulsants / administration & dosage*
  • Anticonvulsants / adverse effects
  • Double-Blind Method
  • Drug Therapy, Combination
  • Follow-Up Studies
  • Humans
  • Infant
  • Placebos
  • Pyridoxine / administration & dosage*
  • Pyridoxine / adverse effects
  • Spasms, Infantile / drug therapy*
  • Thiazines / administration & dosage*
  • Thiazines / adverse effects
  • Treatment Outcome


  • Anticonvulsants
  • Placebos
  • Thiazines
  • sulthiame
  • Pyridoxine