Ohshiro Ikko, Okanishi Tohru, Ohta Ryo, Ohta Kento, Arai Yuto, Kanai Sotaro, Fujimoto Ayataka, Maegaki Yoshihiro
Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.
Neuropediatrics. 2024 Aug;55(4):250-254. doi: 10.1055/a-2298-0747. Epub 2024 Apr 2.
Epileptic spasms without hypsarrhythmia occur when patients do not display hypsarrhythmia on electroencephalogram (EEG) at the onset and throughout the clinical course. We report three patients of epileptic spasms in patients with early onset, all of whom experienced other types of seizures.We detail three patients (two boys and one girl) of epileptic spasms without hypsarrhythmia, occurring between 1 and 3 months of age, with no abnormalities detected on neurometabolic analysis and brain magnetic resonance imaging. Long-term video-EEG monitoring revealed epileptic spasms with focal onset seizures in two patients, and epileptic spasms followed by generalized tonic-clonic seizures in one patient. Hypsarrhythmia was never observed in repeated EEG examinations. Two patients achieved seizure freedom and improved development through treatment with topiramate alone or in combination with valproate, without requiring hormonal therapies or vigabatrin. The remaining patient achieved seizure freedom following administration of antiseizure medications, including topiramate, after a trial of adrenocorticotropic hormone therapy.We report the cases of three patients with early onset epileptic spasms without hypsarrhythmia. All patients achieved seizure freedom after topiramate treatment. Topiramate may be considered as a relatively effective antiseizure medication for early onset epileptic spasms without hypsarrhythmia.
无高峰节律紊乱的癫痫性痉挛发生于患者在发作开始及整个临床过程中脑电图（EEG）未显示高峰节律紊乱时。我们报告了3例早发性癫痫性痉挛患者，他们均经历过其他类型的癫痫发作。我们详细介绍了3例（2名男孩和1名女孩）无高峰节律紊乱的癫痫性痉挛患者，发病年龄在1至3个月之间，神经代谢分析和脑磁共振成像未发现异常。长期视频脑电图监测显示，2例患者癫痫性痉挛发作起始为局灶性发作，1例患者癫痫性痉挛发作后出现全身强直阵挛发作。多次脑电图检查均未观察到高峰节律紊乱。2例患者通过单独使用托吡酯或与丙戊酸联合治疗实现了无癫痫发作且发育改善，无需激素治疗或加巴喷丁。剩余1例患者在促肾上腺皮质激素治疗试验后，使用包括托吡酯在内的抗癫痫药物后实现了无癫痫发作。我们报告了3例早发性无高峰节律紊乱的癫痫性痉挛患者的病例。所有患者经托吡酯治疗后均实现了无癫痫发作。托吡酯可被视为治疗早发性无高峰节律紊乱的癫痫性痉挛的一种相对有效的抗癫痫药物。
