Sulthiame in the Primary Therapy of West Syndrome: A Randomized Double‐blind Placebo‐controlled Add‐on Trial on Baseline Pyridoxine Medication

Abstract

Summary: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 doubleblind 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 One patient in the verum group was withdrawn because of reversible somnolence.Conclusions: As no child in the placebo group responded to the baseline PDX therapy, nor did any child in the STM group during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.

Infantile Spasms

“…33 A recent randomized, placebocontrolled trial of sulthiame began with an open-label, 4-day pyridoxine treatment for all 37 infants. 25 No child in this study responded to pyridoxine. 25 Many neurologists, however, especially in Japan provide vitamin B6 initially as a brief trial, despite this weak evidence, perhaps as a result of its safety and ease of use.…”

“…25 No child in this study responded to pyridoxine. 25 Many neurologists, however, especially in Japan provide vitamin B6 initially as a brief trial, despite this weak evidence, perhaps as a result of its safety and ease of use.…”

Recent Advances in the Pharmacotherapy of Infantile Spasms

“…There are some Class IV studies of topiramate [80][81][82], valproic acid [83,84], Class I of sulthiame [85] and Class IV of zonisamide [86][87][88][89], pyridoxine [90,91], nitrazepam [92], levetiracetam [93], lamotrigine [94] and a ketogenic diet [95,96]. Topiramate is a popular second-line treatment of infantile spasms and is often used as first-line treatment, especially where there is no access to vigabatrin [74] (Class IV).…”

“…B6 was 6/17 versus 0/16 for B6 alone. Adverse effects included hyperventilation, dyspnea, somnolence, paraesthesias, anorexia and psychiatric problems [85] (Class I).…”

Carbonic anhydrase inhibitory properties of novel sulfonamide derivatives of aminoindanes and aminotetralins

“…A carbonic anhydrase (CA) inhibitor drug acetazolamide (diamox, 1) is used to treat glaucoma 2 and idiopathic intracranial hypertension 3 . A sulfonamide drug sultiame (sulthiame, 2) is an anticonvulsant that reported to be used in the treatment of epilepsy and West syndrome 4 . A CA inhibitor diuretic ethoxzolamide (3) can be used in the treatment of glaucoma and duodenal ulcers 5 ( Figure 1).…”