Farwell J, Milstein J, Opheim K, Smith E, Glass S
Epilepsia. 1984 Oct;25(5):605-8. doi: 10.1111/j.1528-1157.1984.tb03469.x.
Infantile spasms constitute a severe seizure disorder unresponsive to standard anticonvulsants. Both prednisone and adrenocorticotropic hormone (ACTH) have produced remission of seizures in some patients. The mechanisms of action of these hormones are not known. Eight infants with infantile spasms were treated with prednisone for 2 weeks. This controlled the seizures in two patients. In the remaining six patients, prednisone was continued and ACTH was added. This treatment produced cessation of spasms in four patients. Serum prednisone and cortisol were measured at a number of points during treatment. In infants receiving prednisone and then prednisone plus ACTH, serum cortisol was suppressed to about one-quarter of baseline levels with the initiation of prednisone, and remained suppressed during ACTH administration. We conclude that ACTH can exert its effect on infantile spasms in the setting of adrenal suppression, and can act without stimulating endogenous cortisol production. A CNS site of action is suggested and should be sought.
婴儿痉挛症是一种对标准抗惊厥药物无反应的严重癫痫疾病。泼尼松和促肾上腺皮质激素(ACTH)在一些患者中都能使癫痫发作缓解。这些激素的作用机制尚不清楚。八名患有婴儿痉挛症的婴儿接受了为期2周的泼尼松治疗。这使两名患者的癫痫发作得到控制。在其余六名患者中,继续使用泼尼松并加用ACTH。这种治疗使四名患者的痉挛停止。在治疗期间的多个时间点测量了血清泼尼松和皮质醇。在接受泼尼松然后泼尼松加ACTH治疗的婴儿中,随着泼尼松的开始使用,血清皮质醇被抑制到基线水平的约四分之一,并在ACTH给药期间一直保持抑制状态。我们得出结论,ACTH在肾上腺抑制的情况下可对婴儿痉挛症发挥作用,且可在不刺激内源性皮质醇产生的情况下发挥作用。提示并应寻找其在中枢神经系统的作用部位。