PDF(Pubmed)
Abstract：
BACKGROUND: Adrenocorticotropic hormone (ACTH) is a tropic hormone naturally secreted by the anterior pituitary gland to stimulate the secretion of cortisol and androgens. ACTH is used in non-tuberous sclerosis infantile epileptic spasms syndrome (IESS), and it has shown significant, promising results in epilepsy syndromes with possible inflammatory processes. However, many studies have also demonstrated a promising potential even in other types of drug-resistant epilepsy. Material and method: This study is a retrospective observational study that follows the clinical characteristics and outcomes of nine pediatric patients with drug-resistant epilepsy treated with short-term synthetic ACTH in Saudi Arabia. The response was assessed during the ACTH infusion and after three months.
RESULTS: During infusion, six of the nine (66%) patients had a short-term (within two weeks) favorable response, with a more than 50% reduction in seizure frequency. Four of the nine (44%) patients had complete responses with seizure freedom. After three months, four patients (44%) had a three-month seizure frequency reduction of more than 30% attributed to ACTH, including one patient with an IESS history who had a 70% reduction in seizure frequency. Of the four patients who had a complete response, three (75%) had a seizure relapse after tapering in the following three months. Conclusion: This case series adds to the literature to suggest ACTH treatment of drug-resistant epilepsies other than IESS might benefit some patients in the acute setting but they are less likely to maintain a sustained treatment response. Randomized and large sample size studies are necessary to assess treatment response and accurately aid in appropriate patient selection.
RESULTS: During infusion, six of the nine (66%) patients had a short-term (within two weeks) favorable response, with a more than 50% reduction in seizure frequency. Four of the nine (44%) patients had complete responses with seizure freedom. After three months, four patients (44%) had a three-month seizure frequency reduction of more than 30% attributed to ACTH, including one patient with an IESS history who had a 70% reduction in seizure frequency. Of the four patients who had a complete response, three (75%) had a seizure relapse after tapering in the following three months. Conclusion: This case series adds to the literature to suggest ACTH treatment of drug-resistant epilepsies other than IESS might benefit some patients in the acute setting but they are less likely to maintain a sustained treatment response. Randomized and large sample size studies are necessary to assess treatment response and accurately aid in appropriate patient selection.
摘要：
背景：促肾上腺皮质激素（ACTH）是垂体前叶自然分泌的一种热带激素，用于刺激皮质醇和雄激素的分泌。ACTH用于非结节性硬化症婴儿癫痫痉挛综合征（IESS），它显示了重要的意义，在可能的炎症过程的癫痫综合征的有希望的结果。然而,许多研究也证明了即使在其他类型的耐药性癫痫中也具有有希望的潜力.材料和方法：本研究是一项回顾性观察性研究，遵循沙特阿拉伯9例接受短期合成ACTH治疗的耐药性癫痫患儿的临床特征和结果。在ACTH输注期间和三个月后评估反应。
结果：在输注期间，9名患者中有6名（66％）有短期（两周内）良好的反应，癫痫发作频率减少50%以上。9名患者中有4名（44％）对癫痫发作无完全反应。三个月后,4例患者（44%）三个月的癫痫发作频率减少超过30%归因于ACTH，包括一名有IESS病史、癫痫发作频率减少70%的患者。在四名有完全反应的患者中，在接下来的三个月中，有3人（75％）的癫痫发作复发。结论：该病例系列增加了文献，表明ACTH治疗除IESS以外的耐药性癫痫可能使某些急性患者受益，但不太可能维持持续的治疗反应。随机和大样本量的研究是必要的，以评估治疗反应和准确地帮助适当的患者选择。
结果：在输注期间，9名患者中有6名（66％）有短期（两周内）良好的反应，癫痫发作频率减少50%以上。9名患者中有4名（44％）对癫痫发作无完全反应。三个月后,4例患者（44%）三个月的癫痫发作频率减少超过30%归因于ACTH，包括一名有IESS病史、癫痫发作频率减少70%的患者。在四名有完全反应的患者中，在接下来的三个月中，有3人（75％）的癫痫发作复发。结论：该病例系列增加了文献，表明ACTH治疗除IESS以外的耐药性癫痫可能使某些急性患者受益，但不太可能维持持续的治疗反应。随机和大样本量的研究是必要的，以评估治疗反应和准确地帮助适当的患者选择。
