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目的 Panayiotopoulos综合征(Panayiotopoulos Syndrome,PS)是一种以自主神经系统症状为主要临床表现的儿童癫痫综合征。本文旨在总结PS的临床及脑电特征。方法收集13例PS患儿的临床、脑电图资料进行分析,部分进行了治疗及随访研究。结果 13例PS患儿,男6例,女7例;起病年龄3岁2个月~10岁8个月。临床以自主神经症状为主,如呕吐、恶心、头痛、面色苍白、腹痛、瞳孔扩大等症状。发作时8例明显意识障碍,4例轻度意识障碍,1例无明显意识障碍。10例发作表现为自主神经持续状态,其中1例发作时间达5h。其余发作形式包括眼球偏斜,部分有眼球偏斜伴头部偏斜。4例出现一侧面部及一侧上下肢抽搐,1例出现全面强直阵挛发作。脑电图特征为所有13例患儿脑电图*清醒期有痫样放电7例,睡眠期均有痫样放电。痫样放电以后头部为主7例,前头部、中央中颞区也可见痫样放电。5例患儿仅出现一次发作。7例口服抗癫痫药物均得到良好控制。发作前后精神发育均正常。结论 PS为儿童良性癫痫,临床发作以自主神经症状为主,持续时间较长,脑电监测以后头部放电为主,预后良好。
Purpose Panayiotopoulos Syndrome (PS) is a childhood epilepsy syndrome characterized by autonomic nervous system symptoms. This article aims to summarize the clinical and EEG features of PS. Methods The clinical data and EEG data of 13 children with PS were collected and analyzed in part for treatment and follow-up. Results 13 cases of PS children, 6 males and 7 females; onset age 3 years 2 months to 10 years 8 months. Clinical main autonomic symptoms, such as vomiting, nausea, headache, pale, abdominal pain, dilated pupils and other symptoms. Eight episodes of episodic apparent disturbance of consciousness, four mild disturbance of consciousness, and one absence of significant disturbance of consciousness. Ten episodes of seizures showed autonomic nervous status, of which one attack time up to 5h. Other forms of seizures, including eye defocus, some with eye deflection with head deflection. 4 cases appeared on one side of the face and one side of the upper and lower extremity convulsions, a case of full tonic clonic seizures. EEG characteristics for all 13 patients EEG * Sense-like discharge in wakefulness in 7 cases, both epileptiform discharge during sleep. Epileptiform discharge after head-based 7 cases, the first head, the central temporal region can also see epileptiform discharge. Five children had only one episode. Seven oral antiepileptic drugs were well controlled. Psychological development before and after the attack were normal. Conclusion PS is a benign epilepsy in children. The main clinical symptom is autonomic nervous symptoms, which lasts longer. The head discharge is mainly after EEG monitoring and the prognosis is good.