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題 名 | Epilepsia Partialis Continua (Kojewnikow's Syndrome): Report of One Case=持續性局部癲癎症(Kojewnikow's症候群):一病例報告 |
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作 者 | 林玲慧; 王本榮; 林明益; 蘇慧琴; 沈友仁; | 書刊名 | 中華民國小兒科醫學會雜誌 |
卷 期 | 31:4 民79.07-08 |
頁 次 | 頁254-261 |
分類號 | 417.5845 |
關鍵詞 | 持續性局部癲癎症; Kojewnikow's症候群; Kojewnikow's syndrome; Epilepsia partialis continua; Myoclonic potential volley; |
語 文 | 英文(English) |
中文摘要 | 持續性局部癲癇症,Kojewnikow's症候羣,是一種相當罕見的癲癇症。主要以長達數小時至數年的持續性局部性肌陣攣來表現。這次我們報告一名,病前極為健康的十二歲男孩,先發作在左軀幹或左下肢的間代性硬直性抽痙後約一個月,出現左大腳趾及其他四趾的獨立性陣攣及左下肢麻痺。左下肢深部腱反射亢進,踝陣攣及病理反射陽性,左下肢肌力輕度減弱。腦波檢查呈正常背景波,在正中心皮野有癲瘸波出現。表面肌電圖一腦波檢查,在左長大腳趾伸肌有明顯的肌陣攣活動電位,但與腦波的正中心皮野的癲病波之間沒有時間上的相關性。我們依其臨床像及電氣生理學檢查的特徵,歸納為Bancaud分類的第一羣,曾檢查頭部電腦斷層、腦血管攝影、核磁共振掃描,以便發現可能的病變,但皆無異常所見。目前病人用抗癲癇劑有部份的改善,但肌陣攣及左下肢輕度麻痺已持續一年六月以上。整個病程中,沒有智能的減退或其他神經病徵的出現。 |
英文摘要 | We describe a 12-year-old boy with EPC (Epilepsia Partialis Continua) who showed well localized myoclonic jerks in the left toes continuing for more than 19 months. Preceding partial clonic or tonic convulsions of the left leg or body were initially noted. Neurological examination disclosed mild muscle weakness of the left leg. During the course, neither mental deterioration nor progressive evolution was noted. Since the EEG revealed well defined midline central spike focus which represent the sensorimotor area of left leg and foot, we postulate that the pathophysiological mechanism of the EPC may be evoked by the focal epileptogenic activation of the motor cortex. EEG-EMG polygraphy proved the well localized myoclonic action potential volleys in the M. extensor hallucis longus of the left great toe. According to the clinical and electrophysiological findings, we classified our case into the group I of Bancaud's definition but failed to find out any demonstrable brain lesion throughout brain CT, MRI and angiography. Neurosurgical intervention is not practical for our case, and medical treatment gave somewhat improvement |
本系統中英文摘要資訊取自各篇刊載內容。