中国神经再生研究(英文版) ›› 2013, Vol. 8 ›› Issue (17): 1623-1630.doi: 10.3969/j.issn.1673-5374.2013.17.011

• 原著:神经损伤修复保护与再生 • 上一篇    

首选多药联合治疗婴儿痉挛

  

  • 收稿日期:2012-12-07 修回日期:2013-03-16 出版日期:2013-06-15 发布日期:2013-06-15

An effective initial polytherapy for children with West syndrome

Feiyong Jia1, Huiyi Jiang1, Lin Du1, Ning Li1, Ji Sun1, Chunbo Niu2   

  1. 1 Department of Pediatric Neurorehabilitation, Second Part of First Hospital, Jilin University, Changchun 130031, Jilin Province, China
    2 Department of Pathology, China-Japan Union Hospital of Jilin University, Changchun 130033, Jilin Province, China
  • Received:2012-12-07 Revised:2013-03-16 Online:2013-06-15 Published:2013-06-15
  • Contact: Chunbo Niu, M.D., Associate professor, Master’s supervisor, Department of Pathology, China-Japan Union Hospital of Jilin University, Changchun 130033, Jilin Province, China, niuchunbo2003@163.com.
  • About author:Feiyong Jia☆, M.D., Associate professor, Master’s supervisor.

摘要:

对婴儿痉挛的治疗的国内外目前多主张首选促肾上腺皮质激素,但仍有大约50%患儿痉挛发作不能得到控制,且有1/3完全控制的患儿复发,不良反应较多。肾上腺皮质激素、妥吡酯、维生素B6和血丙种球蛋白对部分婴儿痉挛有效。文章假设联合应用4种药物可能对婴儿痉挛的治疗具有更好的疗效,并且可能减少药物的不良反应。鉴于此,对新诊断婴儿痉挛患儿51例采用首选联合应用上述4种药物治疗,结果发现治疗后大部分患儿动态脑电图高度节律失常得到明显改善,不良反应明显减少,未出现痉挛发作,复发率低。与症状性患儿相比,隐源性患儿的总疗效率和痉挛无发作率增高,痉挛完全控制时间缩短。结果提示,对婴儿痉挛采用首选促肾上腺皮质激素、妥吡酯、维生素B6、人血丙种球蛋白多药联合治疗的疗效和预后较好,且多药联合治疗隐源性患儿效果优于症状性患儿。

关键词: 神经再生, West 综合征, 痉挛, 病因学, 多药联合治疗, 促肾上腺皮质激素, 妥吡酯, 维生素B6, 静脉用人血丙种球蛋白, 无发作, 脑电图;

Abstract:

Adrenocorticotropic hormone is recommended worldwide as an initial therapy for infantile spasms. However, infantile spasms in about 50% of children cannot be fully controlled by adrenocorticotropic hormone monotherapy, seizures recur in 33% of patients who initially respond to adrenocorticotropic hormone monotherapy, and side effects are relatively common during adrenocorticotropic hormone treatment. Topiramate, vitamin B6, and immunoglobulin are effective in some children with infantile spasms. In the present study, we hypothesized that combined therapy with adrenocorticotropic hormone, topiramate, vitamin B6, and immunoglobulin would effectively treat infantile spasms and have mild adverse effects. Thus, 51 children newly diagnosed with West syndrome including infantile spasms were enrolled and underwent polytherapy with the four drugs. Electroencephalographic hypsarrhythmia was significantly improved in a majority of patients, and these patients were seizure-free, had mild side effects, and low recurrence rates. The overall rates of effective treatment and loss of seizures were significantly higher in cryptogenic children compared with symptomatic children. The mean time to loss of seizures in cryptogenic children was significantly shorter than in symptomatic patients. These findings indicate that initial polytherapy with adrenocorticotropic hormone, topiramate, vitamin B6, and immunoglobulin effectively improves the prognosis of infantile spasms, and its effects were superior in cryptogenic children to symptomatic children.

Key words: neural regeneration, brain injury, West syndrome, spasm, etiology, polytherapy, adrenocorticotropic hormone, topiramate, vitamin B6, intravenous immunoglobulin, seizure-free, electroencephalograph, neuroregeneration