Objectives: The manuscript serves as an update on the current management practices for infantile spasm syndrome (ISS). It includes a detailed summary of the level of current evidence of different treatment options for ISS and gives recommendations for the treatment and care of patients with ISS. Methods: A literature search was performed using the Cochrane and Medline Databases (2014-July 2020). All studies were objectively rated using the Scottish Intercollegiate Guidelines Network. For recommendations, the evidence from these studies was combined with the evidence from studies used in the 2014 guideline. Recommendations: If ISS is suspected, EEG should be performed within a few days and, if confirmed, treatment should be initiated immediately. Response to first-line treatment should be evaluated clinically and electroencephalographically after 14 days. The preferred first-line treatment for ISS consists of either hormone-based monotherapy (ACTH or prednisolone) or a combination of hormone and vigabatrin. Children with tuberous sclerosis complex and those with contraindications against hormone treatment should be treated with vigabatrin. If first-line drugs are ineffective, second-line treatment options such as ketogenic dietary therapies, sulthiame, topiramate, valproate, zonisamide, or benzodiazepines should be considered. Children refractory to drug therapy should be evaluated early for epilepsy surgery, especially if focal brain lesions are present. Parents should be informed about the disease, the efficacy and adverse effects of the medication, and support options for the family. Regular follow-up controls are recommended.

目标：该手稿是对婴儿痉挛综合征 (ISS) 当前管理实践的更新。它详细总结了 ISS 不同治疗方案的当前证据水平，并为 ISS 患者的治疗和护理提供了建议。方法：使用 Cochrane 和 Medline 数据库（2014 年至 2020 年 7 月）进行文献检索。所有研究都使用苏格兰校际指南网络进行了客观评级。对于建议，这些研究的证据与 2014 年指南中使用的研究证据相结合。建议：如果怀疑 ISS，应在几天内进行脑电图检查，如果确诊，应立即开始治疗。14 天后应通过临床和脑电图评估对一线治疗的反应。ISS 的首选一线治疗包括基于激素的单一疗法（ACTH 或泼尼松龙）或激素和氨己烯酸的组合。患有结节性硬化症的儿童和对激素治疗有禁忌症的儿童应使用氨己烯酸治疗。如果一线药物无效，应考虑二线治疗方案，如生酮饮食疗法、硫胺素、托吡酯、丙戊酸盐、唑尼沙胺或苯二氮卓类药物。应对药物治疗无效的儿童进行癫痫手术的早期评估，尤其是在存在局灶性脑损伤的情况下。应告知父母有关疾病、药物的疗效和不良反应以及家庭支持的选择。