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失神發(fā)作相關(guān)的特發(fā)性全面性癲癇綜合征臨床分析

發(fā)布時(shí)間:2018-11-04 18:00
【摘要】:目的:探討失神發(fā)作相關(guān)的特發(fā)性全面性癲癇綜合征臨床特點(diǎn)、腦電圖特征、治療及預(yù)后,為早期正確選擇藥物治療及療程提供依據(jù),早期控制失神發(fā)作可避免認(rèn)知損傷的后果。 方法:對(duì)我院2008年12月至2012年12月兒科癲癇門診及我院神經(jīng)科門診診治的32例與失神發(fā)作相關(guān)的特發(fā)性全面性癲癇綜合征患兒的資料,包括生長(zhǎng)發(fā)育情況、就診年齡、發(fā)作類型、癲癇開始發(fā)作年齡、家族史、腦電圖檢查、影像學(xué)檢查、潛在疾病的診斷、藥物治療等情況進(jìn)行回顧性分析。隨訪時(shí)間1年~3年6個(gè)月。 結(jié)果:32例,男16例,女16例,癲癇開始發(fā)作年齡2歲6月~18歲,平均(9.06±3.86)歲,就診年齡5歲~33歲,,病程1周~15年。以典型失神發(fā)作為唯一發(fā)作形式13例,失神合并肌陣攣發(fā)作3例,失神合并全面強(qiáng)直陣攣發(fā)作4例,同時(shí)有失神、肌陣攣、全面強(qiáng)直陣攣發(fā)作3例,僅有肌陣攣發(fā)作1例,肌陣攣發(fā)作同時(shí)有全面強(qiáng)直陣攣發(fā)作8例。相關(guān)綜合征為兒童失神癲癇13例,青少年失神癲癇5例,青少年肌陣攣癲癇12例,肌陣攣失神癲癇1例,眼瞼肌陣攣伴失神1例。本組神經(jīng)系統(tǒng)查體、頭顱影像學(xué)檢查均正常。有熱性驚厥史5例,癲癇家族史3例。無生后窒息史及外傷史,無其他疾病史。發(fā)病前生長(zhǎng)發(fā)育正常,發(fā)病后認(rèn)知損傷4例。 腦電圖特點(diǎn):32例背景活動(dòng)正常;發(fā)作期腦電圖為雙側(cè)對(duì)稱同步棘慢波、多棘慢波爆發(fā);發(fā)作間期腦電圖可以有局灶性、不規(guī)則和“片段性”放電。過度換氣誘發(fā)失神發(fā)作20例,閃光刺激試驗(yàn)誘發(fā)3例。應(yīng)用抗癲癇藥物治療后,有效29例(總有效率90.6%),發(fā)作完全控制23例(71.9%)。 結(jié)論:本組癲癇綜合征多數(shù)發(fā)生在覺醒期,臨床發(fā)作類型有失神發(fā)作、肌陣攣發(fā)作、全面強(qiáng)直陣攣?zhàn)鳌DX電圖有特征性改變,全導(dǎo)棘慢波/多棘慢波,易被過度換氣、閃光刺激試驗(yàn)所誘發(fā),發(fā)作間期也可見局灶性、不規(guī)律和“片段性”的放電。需要應(yīng)用傳統(tǒng)和新型抗癲癇藥物治療,可以選擇VPA、LEV、LTG、TPM等藥物治療。但需要注意LTG、CBZ、OXC可能會(huì)加重肌陣攣發(fā)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 鹵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.
【學(xué)位授予單位】:吉林大學(xué)
【學(xué)位級(jí)別】:碩士
【學(xué)位授予年份】:2014
【分類號(hào)】:R742.1

【參考文獻(xiàn)】

相關(guān)期刊論文 前1條

1 張平平;張?jiān)氯A;桑田;張鋒;季濤云;黃瓊輝;謝涵;趙海娟;蔡斌;王靜敏;吳曄;吳滬生;許克銘;劉曉燕;陳彪;姜玉武;;染色體15q11.2和15q13.3區(qū)域的微缺失與中國(guó)兒童失神癲沲的相關(guān)性[J];實(shí)用兒科臨床雜志;2012年07期



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