Sher P K, Sheikh M R
Division of Pediatric Neurology, University of Minnesota Medical School, Minneapolis, USA.
Pediatr Neurol. 1993 Nov-Dec;9(6):451-6. doi: 10.1016/0887-8994(93)90024-7.
The records of twenty-six infants with both symptomatic infantile spasms and classic hypsarrhythmia were reviewed to determine the efficacy of various ACTH dosages and time of initiation of therapy. Mean age of infantile spasm onset was 6.4 months. Most patients (13) had sustained perinatal hypoxic-ischemic insults. Seventeen patients (65%) had complete cessation of spasms. Between these responders and the 9 nonresponders there was no difference in duration of spasms prior to treatment (2.6 and 2.0 months) or mean ACTH dose (87.4 and 84.5 U/m2, respectively). Infants treated with high-dose ACTH (> 100 U/m2) did not have an improved response rate. The most favorable outcomes were associated with spasm onset at > 8 months of age (all of whom were responders, regardless of dose) or when treatment was started within 1 month of onset of infantile spasms with > 80 U/m2 ACTH (88% responders). Infants treated more than 2 months after onset often did not respond (57%) regardless of dose. Nonresponders with spasm onset at < 4 months of age had the worst prognoses; all had poorly controlled seizures and regressed developmentally. Although all infants in the study were neurologically abnormal, development either improved or did not deteriorate in most responder infants following spasm resolution and one-half remained seizure free. Nonresponder infants continued to have infantile spasms or other seizure types. These data suggest that ACTH is valuable in the treatment of significantly impaired infants with symptomatic infantile spasms, but the most important determinants of outcome may be age of onset and rapidity of treatment rather than dosage.
回顾了26例有症状性婴儿痉挛症和典型高峰节律紊乱的婴儿记录，以确定不同剂量促肾上腺皮质激素（ACTH）及治疗起始时间的疗效。婴儿痉挛症发作的平均年龄为6.4个月。大多数患者（13例）有围产期持续性缺氧缺血性损伤。17例患者（65%）痉挛完全停止。在这些有反应者和9例无反应者之间，治疗前痉挛持续时间（分别为2.6个月和2.0个月）或平均ACTH剂量（分别为87.4 U/m²和84.5 U/m²）无差异。接受高剂量ACTH（>100 U/m²）治疗的婴儿反应率并未提高。最有利的结果与8个月龄以上出现痉挛有关（无论剂量如何，所有这些婴儿均有反应），或在婴儿痉挛症发作1个月内开始用>80 U/m²的ACTH治疗（88%有反应）。发作后2个月以上开始治疗的婴儿通常无反应（57%），无论剂量如何。4个月龄以下出现痉挛的无反应者预后最差；所有这些婴儿的癫痫发作控制不佳且发育倒退。尽管研究中的所有婴儿均有神经功能异常，但大多数有反应的婴儿在痉挛缓解后发育改善或未恶化，且有一半仍无癫痫发作。无反应的婴儿继续有婴儿痉挛症或其他癫痫发作类型。这些数据表明，ACTH对治疗有严重损伤的有症状性婴儿痉挛症婴儿有价值，但结果的最重要决定因素可能是发作年龄和治疗速度而非剂量。
