Granström M L, Gaily E, Liukkonen E
Epilepsy Unit, Hospital for Children and Adolescents, University of Helsinki, Finland.
Epilepsia. 1999 Jul;40(7):950-7. doi: 10.1111/j.1528-1157.1999.tb00802.x.
The efficacy of a protocol consisting of vigabatrin (VGB) as the first and adrenocorticotropic hormone (ACTH) or valproate (VPA) as the second drug was studied in the treatment of newly diagnosed infantile spasms (IS) during 1994 to 1997 in a population-based design.
Only total disappearance of the spasms with a minimal duration of 1 month was accepted as a response. The treatment response was confirmed by video-EEG study. All infants were studied by magnetic resonance imaging (MRI) or computed tomography (CT) for etiology.
Altogether 42 infants, 10 with cryptogenic and 32 with symptomatic etiology, were treated. Eleven (26%) responded to VGB, five (50%) with cryptogenic, and six (19%) with symptomatic etiology; 91% of infants responded to a dose of 50-100 mg/kg/day, and 82% of them within 1 week. ACTH was offered in combination with VGB to 22 and VPA to four infants for whom VGB failed. Eleven responded to ACTH and one to VPA. In total, 26 (62%) infants responded to the treatment protocol; all (100%) with cryptogenic etiology and 16 (50%) with symptomatic etiology. ACTH treatment was associated with more severe side effects than VGB or VPA. Only one infant relapsed after a spasm-free period with VGB of >4 months, but none after ACTH was combined with VGB.
We suggest VGB as a first drug to all infants with IS. After a treatment trial of 10-14 days with increasing dose from 50 to 150 mg/kg, ACTH should be considered.
在一项基于人群的研究中,对1994年至1997年期间采用以氨己烯酸(VGB)为首选药物、促肾上腺皮质激素(ACTH)或丙戊酸盐(VPA)为次选药物的方案治疗新诊断的婴儿痉挛症(IS)的疗效进行研究。
仅将痉挛完全消失且最短持续1个月视为有效反应。通过视频脑电图研究确认治疗反应。对所有婴儿进行磁共振成像(MRI)或计算机断层扫描(CT)以明确病因。
共治疗42例婴儿,其中10例病因不明,32例有症状性病因。11例(26%)对VGB有反应,5例(50%)病因不明者和6例(19%)有症状性病因者有反应;91%的婴儿对50 - 100 mg/kg/天的剂量有反应,其中82%在1周内有反应。对于VGB治疗无效的22例婴儿联合使用ACTH,4例联合使用VPA。11例对ACTH有反应,1例对VPA有反应。总共26例(62%)婴儿对治疗方案有反应;病因不明者全部(100%)有反应,有症状性病因者16例(50%)有反应。ACTH治疗比VGB或VPA有更严重的副作用。仅1例婴儿在VGB治疗使痉挛停止>4个月后复发,但ACTH与VGB联合使用后无复发。
我们建议对所有IS婴儿首选VGB。在以50至150 mg/kg递增剂量进行10 - 14天的治疗试验后,应考虑使用ACTH。