Echenne B, Dulac O, Parayre-Chanez M J, Chiron C, Taillebois L, Cognot C, Andary M, Clot J, Baldy-Moulinier M
Unité de Neuropédiatrie, Centre Gui de Chauliac, Montpellier, France.
Brain Dev. 1991 Sep;13(5):313-9. doi: 10.1016/s0387-7604(12)80125-2.
In a prospective study, 23 children with infantile spasms received intravenous gammaglobulins in high doses. 19 patients present a West syndrome. 4 older patients were included in the study because infantile spasms had preceded their Lennox-Gastaut syndrome. None of the patients had recently undergone corticosteroid therapy. No effect was observed in 15 patients, while transitory clinical and/or electroencephalographic improvement was noted in 3. Complete normalization was obtained in the remaining 5 patients, of whom 4 had severe brain lesions. No correlation existed between the therapeutic results and immunological abnormalities, a deficiency in IgG subclasses in particular. On the whole, the therapeutic results were disappointing. But the existence of some cases in which spectacular electroencephalographic and/or clinical improvement was obtained leads us to suggest that IV gamma-globulins be used as auxiliary treatment in infantile spasms.
在一项前瞻性研究中,23名患有婴儿痉挛症的儿童接受了大剂量静脉注射丙种球蛋白治疗。19例患者呈现韦斯特综合征。4名年龄较大的患者被纳入研究,因为婴儿痉挛症先于他们的伦诺克斯-加斯东综合征出现。所有患者近期均未接受过皮质类固醇治疗。15例患者未观察到效果,3例患者出现了短暂的临床和/或脑电图改善。其余5例患者实现了完全正常化,其中4例有严重脑损伤。治疗结果与免疫异常之间不存在相关性,尤其是IgG亚类缺乏。总体而言,治疗结果令人失望。但存在一些脑电图和/或临床得到显著改善的病例,这使我们建议将静脉注射丙种球蛋白用作婴儿痉挛症的辅助治疗。