Buoni Sabrina, Zannolli Raffaella, Strambi Mirella, Fois Alberto
Department of Pediatrics, Obstetric and Reproductive Medicine, Policlinico Le Scotte, University of Siena, Italy.
J Child Neurol. 2004 May;19(5):385-6. doi: 10.1177/088307380401900512.
The clinical and electroencephalographic (EEG) response to combined therapy with vigabatrin and topiramate was evaluated in five patients ages 7 to 15 months affected by West syndrome in an open-label trial. Four patients had cryptogenic and one patient had symptomatic (tuberous sclerosis) West syndrome. In cryptogenic patients who failed to respond to pyridoxine, vigabatrin was titrated to 80 to 100 mg/kg. Because control of infantile spasms or an EEG improvement was not obtained with vigabatrin treatment, topiramate was added (3-3.8 mg/kg/day). In all patients, the combined therapy with topiramate and vigabatrin achieved a rapid and complete normalization of infantile spasms, and in three patients with cryptogenic West syndrome, the EEG also became normal. In only one patient, transient anorexia was observed. This drug combination led to rapid neurodevelopmental normalization in cryptogenic patients. The results are promising and justify more trials in larger numbers of children with West syndrome.
在一项开放标签试验中，对5名年龄在7至15个月的患有韦斯特综合征的患儿进行了评估，以观察加巴喷丁和托吡酯联合治疗的临床及脑电图（EEG）反应。4名患儿为隐源性韦斯特综合征，1名患儿为症状性（结节性硬化症）韦斯特综合征。对于对吡哆醇无反应的隐源性患儿，加巴喷丁的剂量逐渐滴定至80至100mg/kg。由于加巴喷丁治疗未能控制婴儿痉挛或改善脑电图，因此加用了托吡酯（3 - 3.8mg/kg/天）。在所有患儿中，托吡酯和加巴喷丁联合治疗使婴儿痉挛迅速且完全缓解，3名患有隐源性韦斯特综合征的患儿脑电图也恢复正常。仅1名患儿出现短暂性厌食。这种药物组合使隐源性患儿的神经发育迅速恢复正常。结果很有前景，有必要在更多韦斯特综合征患儿中进行更多试验。
