丙戊酸与促肾上腺皮质激素作为婴儿痉挛症一线治疗的比较：一项随机、前瞻性研究。

Vigabatrin versus ACTH as first-line treatment for infantile spasms: a randomized, prospective study.

作者信息

Vigevano F, Cilio M R

机构信息

Department of Child Neurology, Bambino Gesù Children's Hospital, Rome, Italy.

出版信息

Epilepsia. 1997 Dec;38(12):1270-4. doi: 10.1111/j.1528-1157.1997.tb00063.x.

DOI:10.1111/j.1528-1157.1997.tb00063.x

PMID:9578521

Abstract

PURPOSE

To compare the efficacy and tolerability of vigabatrin (VGB) and adrenocorticotrophic hormone (ACTH) as first-line therapy in infantile spasms.

METHODS

Forty-two infants (22 males, 20 females) aged 2-9 months with newly diagnosed infantile spasms, were included in the trial. Patients were randomized to receive VGB 100-150 mg/kg/day or Depot ACTH 10 IU/day. The alternative drug was given if spasms were not controlled within 20 days or in cases of intolerance to initial therapy. Twenty-three patients (7 cryptogenic, 16 symptomatic) received VGB as first-line therapy; 19 patients (8 cryptogenic, 11 symptomatic) received ACTH as the first drug.

RESULTS

Cessation of spasms was observed in 11 (48%) of the patients randomized to VGB and in 14 (74%) of those randomized to ACTH. Response to VGB was observed within 1-14 days, but two-thirds of patients (7/11) responded within 3 days. In the group treated with VGB, side effects such as drowsiness, hypotonia and irritability were observed in 13% of patients, compared with 37% in the group treated with ACTH. VGB was more effective than ACTH as treatment for cerebral malformations or tuberous sclerosis, whereas ACTH proved more effective in perinatal hypoxic/ischemic injury. The efficacy of the two drugs was similar in cryptogenic cases. Disappearance of interictal EEG abnormalities occurred sooner in patients randomized to ACTH than in those who received VGB as initial therapy. During the second phase, the alternative drug was given to the resistant patients. Spasms ceased in 2 of 5 patients treated with VGB and in 11 of 12 patients treated with ACTH. After 3 months, relapses of spasms were observed in 6 patients treated with ACTH and in 1 treated with VGB. VGB produced a therapeutic response in nearly half the patients receiving this drug.

CONCLUSIONS

Our data lend further support to the view that VGB may be considered a first-choice drug in the treatment of IS.

摘要

目的

比较氨己烯酸（VGB）和促肾上腺皮质激素（ACTH）作为婴儿痉挛症一线治疗药物的疗效和耐受性。

方法

42例年龄在2至9个月、新诊断为婴儿痉挛症的婴儿（男22例，女20例）纳入试验。患者随机接受VGB 100 - 150mg/kg/天或长效ACTH 10IU/天治疗。若痉挛在20天内未得到控制或出现对初始治疗不耐受的情况，则给予替代药物。23例患者（7例隐源性、16例症状性）接受VGB作为一线治疗；19例患者（8例隐源性、11例症状性）接受ACTH作为首选药物。

结果

随机接受VGB治疗的患者中有11例（48%）痉挛停止，随机接受ACTH治疗的患者中有14例（74%）痉挛停止。VGB治疗后1至14天出现反应，但三分之二的患者（7/11）在3天内出现反应。在接受VGB治疗的组中，13%的患者出现嗜睡、肌张力减退和易激惹等副作用，而接受ACTH治疗的组中这一比例为37%。VGB对脑畸形或结节性硬化症的治疗效果优于ACTH，而ACTH在围产期缺氧缺血性损伤方面被证明更有效。两种药物在隐源性病例中的疗效相似。随机接受ACTH治疗的患者发作间期脑电图异常消失的时间比接受VGB初始治疗的患者更早。在第二阶段，对耐药患者给予替代药物。接受VGB治疗的5例患者中有2例痉挛停止，接受ACTH治疗的12例患者中有11例痉挛停止。3个月后，接受ACTH治疗的6例患者和接受VGB治疗的1例患者出现痉挛复发。VGB在近一半接受该药物治疗的患者中产生了治疗反应。