失神发作相关的特发性全面性癫痫综合征临床分析

发布时间：2018-11-04 18:00

【摘要】：目的：探讨失神发作相关的特发性全面性癫痫综合征临床特点、脑电图特征、治疗及预后，为早期正确选择药物治疗及疗程提供依据，早期控制失神发作可避免认知损伤的后果。方法：对我院2008年12月至2012年12月儿科癫痫门诊及我院神经科门诊诊治的32例与失神发作相关的特发性全面性癫痫综合征患儿的资料，包括生长发育情况、就诊年龄、发作类型、癫痫开始发作年龄、家族史、脑电图检查、影像学检查、潜在疾病的诊断、药物治疗等情况进行回顾性分析。随访时间1年～3年6个月。结果：32例，男16例，女16例，癫痫开始发作年龄2岁6月～18岁，平均（9.06±3.86）岁，就诊年龄5岁～33岁，，病程1周～15年。以典型失神发作为唯一发作形式13例，失神合并肌阵挛发作3例，失神合并全面强直阵挛发作4例，同时有失神、肌阵挛、全面强直阵挛发作3例，仅有肌阵挛发作1例，肌阵挛发作同时有全面强直阵挛发作8例。相关综合征为儿童失神癫痫13例，青少年失神癫痫5例，青少年肌阵挛癫痫12例，肌阵挛失神癫痫1例，眼睑肌阵挛伴失神1例。本组神经系统查体、头颅影像学检查均正常。有热性惊厥史5例，癫痫家族史3例。无生后窒息史及外伤史，无其他疾病史。发病前生长发育正常，发病后认知损伤4例。脑电图特点：32例背景活动正常；发作期脑电图为双侧对称同步棘慢波、多棘慢波爆发；发作间期脑电图可以有局灶性、不规则和“片段性”放电。过度换气诱发失神发作20例，闪光刺激试验诱发3例。应用抗癫痫药物治疗后，有效29例（总有效率90.6%），发作完全控制23例（71.9%）。结论：本组癫痫综合征多数发生在觉醒期，临床发作类型有失神发作、肌阵挛发作、全面强直阵挛作。脑电图有特征性改变，全导棘慢波/多棘慢波，易被过度换气、闪光刺激试验所诱发，发作间期也可见局灶性、不规律和“片段性”的放电。需要应用传统和新型抗癫痫药物治疗，可以选择VPA、LEV、LTG、TPM等药物治疗。但需要注意LTG、CBZ、OXC可能会加重肌阵挛发作。
[Abstract]:Objective: to investigate the clinical features, electroencephalogram (EEG), treatment and prognosis of idiopathic generalized epilepsy syndrome associated with aphasia, so as to provide evidence for early and correct choice of drug therapy and course of treatment. Early control of aphasia can avoid the consequences of cognitive impairment. Methods: from December 2008 to December 2012, 32 children with idiopathic comprehensive epilepsy syndrome associated with aphasia, including growth and development, age of visit, were collected from pediatric epilepsy clinic and neurology clinic in our hospital. The types of seizures, onset age, family history, EEG, imaging, diagnosis of underlying diseases, and drug therapy were retrospectively analyzed. The follow-up time was 1 year to 3 years and 6 months. Results: there were 32 cases (16 males and 16 females) with onset of epilepsy from 2 to 18 years old (mean (9.06 卤3.86) years). The patients were 5 to 33 years old and the course of disease was 1 week to 15 years. There were 13 cases with typical aphasia, 3 cases with myoclonic seizure, 4 cases with total tonic-clonic seizure, 3 cases with total tonic-clonic seizure, and 1 case with myoclonic seizure. Myoclonic seizures were also found in 8 patients with generalized tonic-clonic seizures. The related syndromes were childhood aphasia in 13 cases, juvenile aphasia in 5 cases, juvenile myoclonic epilepsy in 12 cases, myoclonic aphasia in 1 case, eyelid myoclonus with aphasia in 1 case. The neurological examination and head imaging were all normal. There were 5 cases of febrile convulsion and 3 cases of epileptic family history. No history of postnatal asphyxia and trauma, no history of other diseases. Growth and development were normal before onset and cognitive impairment occurred in 4 cases. The EEG features were as follows: normal background activity in 32 cases, bilateral symmetric synchronous spike and slow wave in attack, and localized, irregular and "piecewise" discharges in interictal EEG. Hyperventilation induced aphasia in 20 cases and flash stimulation test in 3 cases. After treatment with antiepileptic drugs, 29 cases were effective (total effective rate 90.6%), 23 cases (71.9%) had complete seizure control. Conclusion: most of the epileptic syndrome occurred in the period of arousal. The clinical seizure types were aphasia, myoclonic seizure and total tonic-clonic seizure. Electroencephalogram (EEG) is characterized by slow / multi-spike slow waves, which are easily induced by excessive ventilation, flash stimulation test, and localized, irregular and "piecewise" discharges during the interictal period. Traditional and new antiepileptic drugs need to be used, VPA,LEV,LTG,TPM and other drugs can be chosen. Note, however, that LTG,CBZ,OXC may aggravate myoclonic seizures.
【学位授予单位】：吉林大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R742.1

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