合併有癲癇性障礙的特殊性語言發展障礙－個案報告

Specific Language Impairment Associated with Epilepsy-Case Report

10.6143/JSLHAT.2013.06.04

尹丹桂(Tan-Kuai Wan)；王拔群(Pa-Chun Wang)；劉勻智(Yun-Chih Liu)；陳岱靈(Tai-Ling Chen)；洪焜隆(Kun-Long Hung)

藍道克利夫症（後天性癲癇性失語症） ； 癲癇引起的突發語言退化 ； 中樞聽覺處理異常 ； Landau Kleffner syndrome (acquired epleptic aphasia) ； Sudden onset of language impairment induced by epilepsy ； Central auditory processing disorder

台灣聽力語言學會雜誌

30期（2013 / 06 / 01）

57 - 64

繁體中文

兒童在臨床上出現癲癇的症狀，合併睡眠時腦部異常放電，造成認知及語言退化的案例並不罕見，在癲癇及癲癇性症候群之國際分類中，某些癲癇併有睡眠時腦波異常放電的症候群目前被歸類在未確定之局部或泛發性之癲癇及症候群中這一項，當中比較多被提出來討論及比較的是癲癇伴有慢波睡眠時出現持續性棘慢波（Continuous Spikes and Waves During Sleep，CSWS）及後天性癲癇症性失語症，又名藍道克利夫症（Landau Kleffner Syndrome，LKS）。本病例報告主要提出一名八歲的病患，智能體格發育正常，在三歲多時被發現語言發展較遲緩且過動，曾接受過一段時間的治療；但在去年開始被陸續觀察到疑似有癲癇發作，同時語言及行為能力突然退化，臨床表現為有時候聽不到或聽不懂別人的指令，也無法正確回應，先到小兒神經內科就診後，被轉介到耳鼻喉科作進一步檢查；純音聽力檢查（Pure ToneAudiometry，PTA）異常，與語音接收閥值（Speech Reception Threshold，SRT）不符，遂安排腦幹聽性反應（Auditory Brainstem Response，ABR）及變頻耳聲傳射（Distortion Product Otoacoustic Emission，DPOAE）檢查，顯示結果正常，不能排除病患是否因為理解有問題或是無法配合所造成PTA結果異常，並非真正的週邊性聽力障礙；腦波檢查顯示左側顳葉局部放電，睡眠期更出現連續放電現象，因此被懷疑是LKS。由於LKS很罕見，病理機轉目前還不清楚，因此在LKS被正確診斷之前，需要花費一些時間，耳鼻喉科醫師及聽力師要有高度警覺性，而目前耳鼻喉科文獻回顧中沒有提出典型或標準的判讀方式。本病例經專家意見討論認為還無法完全符合LKS的診斷標準，只能診斷為癲癇引起的突發語言退化（sudden onset of language impairment induced by epilepsy），診斷及治療過程有許多值得學習檢討處，特提出案例報告。

Various childhood epileptic syndromes associated with dramatic activation of the epileptiform activities during slow wave sleep may manifest with progressive psychomotor decline, which cannot be otherwise attributed to known metabolic or organic causes. The main representatives are the continuous spike and waves during slow wave sleep syndrome (CSWS) and the Landau-Kleffner syndrome (LKS). LKS is a rare childhood-acquired aphasia in conjunction with epilepsy. It typically occurs in children between the ages of 3 and 10 with normal developmental milestone. Onset can be abrupt or insidious. Initial presentation is mainly an auditory processing difficulty known as verbal auditory agnosia. Diagnosis can be confirmed with typical electroencephalographyic findings. The EEG during sleep demonstrates persistent convulsive discharges known as electrical status epilepticus during slow wave sleep (ESES) which results in receptive and expressive language deterioration. Clinical manifestations are highly variable and diagnosis is often delayed. Here we present a case of an 8-year-old boy whose development appeared to be normal until the sudden onset of language impairment and seizure early this year. Audiometry showed fluctuating pure tone threshold but normal ABR and DPOAE. Sleep EEG showed repeated high amplitude sharp and spikes very similar to ESES. We present this case with ”sudden onset of language impairment induced by epilepsy” for important differential diagnosis of Landau-Kleffner syndrome. Since the first presentation of language problem is often word deafness or auditory agnosia, otolaryngologists and audiologists tend to be initially involved and should be aware of this disorder.

1. Great Ormond Street Hospital for Children NHS Trust. (2005). Landau Kleffner Syndrome (LKS): In-formation for families. Retrieved September 5, 2012, from http://www.friendsoflks.com/LKSInfo.pdf
2. Arts, W. F.,Aarsen, F. K.,Scheltens-de Boer, M.,Catsman-Berrevoets, C. E.(2009).Landau-Kleffner syndrome and CSWS syndrome: Treatment with intravenous immunoglobulins.Epilepsia,50,55-58.
3. Bellis, T. J.(1996).Assessment and management of central auditory processing disorders in the educational setting from science to practice.San Diego, CA:Singular.
4. Billard, C.,Fluss, J.,Pinton, F.(2009).Specific language impairment versus Landau-Kleffner syndrome.Epilepsia,50,21-24.
5. Cross, J. H.,Neville, B. G.(2009).The surgical treatment of Landau-Kleffner syndrome.Epilepsia,50,63-67.
6. Fandiño, M.,Connolly, M.,Usher, L.,Palm, S.,Kozak, F. K.(2011).Landau-Kleffner syndrome: A rare auditory processing disorder series of cases and review of the literature.International Journal of Pediatric Otorhinolaryngology,75,33-38.
7. 趙可屏、曾涼芬、趙文崇(1998)。語音聽覺缺失症。聽語會刊，13，100-109。