National Epilepsy Center, NHO Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan.
Department of Pediatrics, Gifu University School of Medicine, Gifu, Japan.
Epilepsia Open. 2022 Mar;7(1):85-97. doi: 10.1002/epi4.12560. Epub 2021 Nov 19.
Among standard treatments for infantile spasms, adrenocorticotropic hormone (ACTH) is reported as the best treatment, but ACTH is ineffective in one-half of the patients. To establish precision medicine, we examined pharmacoresistance of focal epileptic spasms (ES), generalized ES, and generalized ES combined with focal seizures, diagnosed based on the revised seizure classification of ILAE in 2017.
We conducted a retrospective nationwide study in Japan on the long-term seizure outcome of ES. Long-term seizure outcome was evaluated by seizure-free rate, seizure-free period, and Kaplan-Meier curve. Seizure-free was defined as seizure control for longer than 2 months.
From the medical history of 501 patients, 325 patients had generalized ES only (GES group) at the start of the first treatment, 125 patients had generalized ES after focal seizure onset (FS-GES group), seven patients had focal ES after focal seizure onset (FS-FES group), and 24 patients had generalized ES combined with focal seizures after focal seizure onset (FS-GES + FS group). Seizure-free period of ES (generalized ES and focal ES) [mean (95% confidence interval)] was 2.7 (0.0-5.4) months in GES group, 1.1 (0.1-2.2) months in FS-GES group, 1.0 (0.2-1.9) months in FS-GES + FS group, and 0.1 (-0.2-0.5) months in FS-FES group. Seizure-free rate, seizure-free period, and Kaplan-Meier curve of generalized ES were almost the same in GES group and FS-GES group, with characteristics of superior response to ACTH. Mean seizure-free period of generalized ES combined with focal seizures was significantly shorter in FS-GES + FS group than in GES group. Mean seizure-free period of focal ES in FS-FES group was extremely short with exceedingly early relapse.
Pharmacoresistance was different in generalized ES, focal ES, and generalized ES combined with focal seizures. ES with focal features or with focal seizures may have focal lesions, thus consider surgical options earlier in the course.
在婴儿痉挛症的标准治疗中，促肾上腺皮质激素（ACTH）被报道为最佳治疗方法，但有一半的患者对此治疗无效。为了建立精准医学，我们根据 2017 年 ILAE 修订的癫痫发作分类，检查了局灶性癫痫痉挛（ES）、全面性 ES 和全面性 ES 合并局灶性发作的药物反应。
我们在日本进行了一项回顾性全国性研究，以评估 ES 的长期癫痫发作结局。通过无癫痫发作率、无癫痫发作期和 Kaplan-Meier 曲线评估无癫痫发作。无癫痫发作定义为控制癫痫发作超过 2 个月。
从 501 名患者的病史中，325 名患者在开始首次治疗时仅有全面性 ES（GES 组），125 名患者在局灶性发作后出现全面性 ES（FS-GES 组），7 名患者在局灶性发作后出现局灶性 ES（FS-FES 组），24 名患者在局灶性发作后出现全面性 ES 合并局灶性发作（FS-GES+FS 组）。ES（全面性 ES 和局灶性 ES）的无癫痫发作期（中位数（95%置信区间））在 GES 组中为 2.7（0.0-5.4）个月，在 FS-GES 组中为 1.1（0.1-2.2）个月，在 FS-GES+FS 组中为 1.0（0.2-1.9）个月，在 FS-FES 组中为 0.1（-0.2-0.5）个月。GES 组和 FS-GES 组的全面性 ES 的无癫痫发作率、无癫痫发作期和 Kaplan-Meier 曲线几乎相同，对 ACTH 的反应较好。FS-GES+FS 组的全面性 ES 合并局灶性发作的平均无癫痫发作期明显短于 GES 组。FS-FES 组局灶性 ES 的平均无癫痫发作期极短，复发较早。
全面性 ES、局灶性 ES 和全面性 ES 合并局灶性发作的药物反应不同。具有局灶性特征或局灶性发作的 ES 可能有局灶性病变，因此在病程早期更早考虑手术选择。
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