■尽管有许多指导方针,婴儿痉挛的总体结局很差,只有少数患者能够上学。这项研究的目的是调查长期结果。患者难以获得推荐的一线抗癫痫药物(ASM),如激素(促肾上腺皮质激素或泼尼松龙/泼尼松)和vigabatrin,他们的替代疗法是其他ASM和生酮饮食。
■在2014年1月至2022年8月之间在电子病历系统中至少有2年病历的婴儿痉挛患者被纳入本研究。患者资料进行回顾性分析。所有患者均接受了生酮饮食疗法(主要是经典的生酮饮食疗法)。生酮饮食疗法与不用作一线疗法的ASM组合。主要终点结果测量是癫痫发作自由的患者人数。次要措施包括生酮饮食治疗的持续时间,ASM的选择,以及最后一次就诊时的患者发育。
■共包括177例婴儿痉挛患者,其中152人(86%)有癫痫发作自由。从第一次到最后一次住院的中位时间为53.27个月,访问次数为47.00。初次住院时的平均年龄为8.00个月,开始生酮饮食的中位年龄为17.73个月.在最后一次访问中,神经发育迟缓患者的比例,发展性癫痫脑病,耐药癫痫,和广泛的癫痫发作显著增加。常用的ASM是托吡酯,丙戊酸,左乙拉西坦,硝西泮,和维生素B6注射,而推荐的一线药物促肾上腺皮质激素和vigabatrin很少被选择。9.5年的研究时间分为三个时期,但ASM的处方在这些时期之间没有显着变化。
■尽管使用生酮饮食疗法结合非标准ASM,癫痫发作自由率很高,患者在最后一次就诊时出现了明显的神经发育迟缓,那是,然而,类似于标准治疗。为了改善婴儿痉挛的结果,需要将生酮饮食与非标准ASM联合作为一线治疗的多中心临床试验。
UNASSIGNED: Despite numerous guidelines, the overall outcome of infantile spasms is poor, with only a small number of patients being able to attend school. The purpose of this study was to investigate long-term outcomes. Patients had poor access to the recommended first-line anti-seizure medications (ASMs), such as hormones (corticotropin or prednisolone/prednisone) and vigabatrin, and their alternative treatment was other ASMs and a ketogenic diet.
UNASSIGNED: Patients suffering from infantile spasms who had at least 2 years of medical records in the electronic medical record system between January 2014 and August 2022 were included in this study. Patient information was retrospectively reviewed. All patients had received ketogenic diet therapy (mainly classical ketogenic diet therapy). The ketogenic diet therapy was combined with ASMs not used as first-line therapies. The primary endpoint outcome measure was the number of patients with seizure freedom. The secondary measures included the duration of ketogenic diet therapy, choice of ASMs, and patient development at the last visit.
UNASSIGNED: A total of 177 patients with infantile spasms were included, and 152 (86%) of them had seizure freedom. The median duration from the first to the last hospital visit was 53.27 months, and the number of visits was 47.00. The median age at the initial hospital visit was 8.00 months, and the median age at initiation of the ketogenic diet was 17.73 months. At the last visit, the proportions of patients with neurodevelopmental delay, developmental epileptic encephalopathy, drug-resistant epilepsy, and generalized seizures increased significantly. The frequently used ASMs were topiramate, valproic acid, levetiracetam, nitrazepam, and vitamin B6 injection, while the recommended first-line drugs corticotropin and vigabatrin were rarely selected. The study duration of 9.5 years was divided into three periods but the prescription of ASMs did not change significantly between these periods.
UNASSIGNED: Although the seizure freedom rate was high with ketogenic diet therapy combined with non-standard ASMs, the patients had a significant neurodevelopmental delay at the last visit, which was, however, similar to that of standard treatment. To improve the outcomes of infantile spasms, multicenter clinical trials of the ketogenic diet as a first-line treatment in combination with non-standard ASMs are needed.