Ikegami Mariko, Takahashi Yukitoshi, Ikeda Hiroko, Imai Katsumi, Otani Hideyuki, Kubota Yuko, Shigematsu Hideo, Takayama Rumiko, Mogami Yukiko
Department of Pediatrics, National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorder, Shizuoka.
No To Hattatsu. 2013 Jul;45(4):281-7.
We examined the effectiveness of repeated adrenocorticotropic hormone (ACTH) therapy in short-term and long-term seizure control in patients with intractable epileptic spasms.
Twenty-five patients with intractable spasms, in whom epileptic seizures were not controlled or relapsed after the first ACTH therapy, were given repeated ACTH therapy. The short-term effect (seizure control longer than two months) of repeated ACTH therapy was analyzed, and the long-term effect was estimated by Kaplan-Meier method.
Short-term seizure control by repeated ACTH therapy was achieved in 13 of 25 patients (52.0%), and in 5 of 13 patients, seizures were controlled by ACTH therapy at higher doses compared with the first ACTH therapy. Short-term effectiveness was obtained in 76.5% of patients who had epileptic spasms alone at the time of the second ACTH therapy, but was ineffective in all 8 patients who had multiple types of seizures, with relapses within 2 months. Short-term effectiveness was not associated with clinical factors such as onset age, age of repeated ACTH treatment, and EEG findings. Regarding the long-term effect of repeated ACTH therapy, the period until seizure relapse was significantly longer in patients with epileptic spasms alone compared to patients with multiple seizure types. Spasms were controlled in 5 of 25 cases (20.0%) at the final observation. In patients with multiple seizure types and patients with onset age older than eight months, seizure control was not obtained. Long-term outcome was good in patients with treatment lag within 2 months.
In repeated ACTH therapy, seizure type seems to be one of the major determinants for short- and long-term seizure outcome.
我们研究了重复促肾上腺皮质激素（ACTH）治疗对难治性癫痫痉挛患者短期和长期癫痫控制的有效性。
25例难治性痉挛患者，在首次ACTH治疗后癫痫发作未得到控制或复发，接受了重复ACTH治疗。分析重复ACTH治疗的短期效果（癫痫发作控制超过两个月），并采用Kaplan-Meier法评估长期效果。
25例患者中有13例（52.0%）通过重复ACTH治疗实现了短期癫痫控制，其中13例患者中有5例，与首次ACTH治疗相比，更高剂量的ACTH治疗控制了癫痫发作。在第二次ACTH治疗时仅患有癫痫痉挛的患者中，76.5%获得了短期疗效，但在所有8例患有多种类型癫痫发作且在2个月内复发的患者中均无效。短期疗效与发病年龄、重复ACTH治疗年龄和脑电图结果等临床因素无关。关于重复ACTH治疗的长期效果，仅患有癫痫痉挛的患者癫痫复发的时间明显长于患有多种癫痫发作类型的患者。在最后一次观察时，25例中有5例（20.0%）痉挛得到控制。在患有多种癫痫发作类型且发病年龄超过8个月的患者中，未实现癫痫控制。治疗延迟在2个月内的患者长期预后良好。
在重复ACTH治疗中，癫痫发作类型似乎是短期和长期癫痫发作结果的主要决定因素之一。
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