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目的 分析和探讨大田原综合征 (OS)的病因、临床、EEG、神经影像学和转归的特征以及与其他年龄依赖性癫疒间 脑病的关系。方法 通过住院观察及EEG、脑干诱发电位、CT、MRI、血生化等辅助检查和随访 ,对 17例OS的诊治进行分析和探讨。结果 17例中 ,男 11例 ,女 6例 ;首发年龄 :生后 8h至80d ,其中 <1个月 11例 ,平均 2 7d ;所有患儿都有强直或 (和 )强直痉挛发作史 ,多数伴有部分性或非对称性痉挛发作 ,每日发作 10～ 5 0次不等 ;同时伴有严重的神经精神发育迟滞 ;母孕史和家族史异常 9例 ,窒息史 4例 ;影像学显示非对称性脑发育不全 9例 ,双侧广泛性脑萎缩伴外部性脑积水 7例 ;发作间期EEG为暴发抑制图形 ,发作期表现为高幅慢波和不规则棘波或棘慢波与低波幅快波活动交替。结论 OS是年龄依赖性癫疒间 脑病中最严重的早期婴儿癫疒间 综合征 ,与多种病因有关 ;预后差 ,与WS和LGS具有年龄依赖性演变的相互关系。
Objective To analyze and discuss the etiology, clinical, EEG, neuroimaging and prognosis of Haodahara syndrome (OS) and its relationship with other age-dependent epileptic encephalopathy. Methods The diagnosis and treatment of 17 cases of OS were analyzed and analyzed through hospitalization observation, EEG, brain stem evoked potentials, CT, MRI and blood biochemistry. Results In 17 cases, there were 11 males and 6 females. The starting age was from 8 hours to 80 days after birth, of which 11 cases <1 month, an average of 27 days. All patients had a history of ankylosing spasms and / or tonic spasms, Accompanied by partial or asymmetric spasms of seizures, 10 to 50 times a day attack; accompanied by severe neuropsychiatric lag; mother’s pregnancy and family history of abnormalities in 9 cases, 4 cases of asphyxia; imaging showed 9 cases of asymmetric brain hypoplasia, bilateral extensive brain atrophy with external hydrocephalus in 7 cases; interictal EEG outbreak suppression pattern, the attack showed high amplitude slow wave and irregular spike or spike Alternate with low amplitude fast wave activity. Conclusions OS is the most serious form of epileptic seizures in age-dependent epileptic encephalopathy and is associated with a variety of causes; the poor prognosis is associated with an age-dependent evolution of WS and LGS.