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題 名 | Centronuclear Myopathy with Unusual Presentation of Electrical Myotonia and Supraventricular Tachycardia: Report of a Case=中心核肌病變:一病例報告 |
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作 者 | 尹居浩; 許耀東; 李俊泰; | 書刊名 | 醫學研究 |
卷 期 | 20:6 2000.06[民89.06] |
頁 次 | 頁308-315 |
分類號 | 415.3165 |
關鍵詞 | 中心核肌病變; 肌強直症; 心肌病變; 心室上早發性收縮; Centronuclear myopathy; Myotonia; Cardiomyopathy; Supraventricular tachycardia; |
語 文 | 英文(English) |
中文摘要 | 中心核肌病變很少合併心肌病變。本文報告一名二十歲年輕男性,臨床表現頭髮微禿、面部肌肉無力、兩側眼瞼下垂、以及頸部、肢體肌肉萎縮無力。當其產生心悸時,心電圖偵測到心室上早發性收縮。心臟超音波顯示僧帽瓣增厚及脫垂、左心室汎發性運動不良及收縮功能不良。肌電圖顯示類肌強直波。肌肉病理切片檢查顯現於主要的兩型肌纖維具有高比例的中心核,且第一型肌纖維萎縮。本病例合併以心室上早發性收縮為特徵之心肌病變及明顯的電器性肌強直,甚屬罕見,更可能因為其肌強直波與心律不整,而被誤診為失養性肌強直症。 |
英文摘要 | Centronuclear myopathy is associated infrequently with cardiomyopathy. We present a sporadic case of a 20-year-old young male with mild frontal baldness, facial weakness, and bilateral ptosis, as well as weakness and muscle wasting involving the neck, limb girdles and limbs. ECG showed paroxysmal supraventricular tachycardia during palpitation episode. Echocardiography showed prolapse as well as thickness of anterior leaflet of mitral valve, left ventricle generalized hypokinesia, and mild left ventricular systolic dysfunction. EMG study displayed myotonia-like discharges. Muscle biopsy showed high frequency of central nuclear fibres in both main fibre types and type 1 fibre atrophy. We conclude that myotonia-like discharges on the EMG and cardiac arrhythmia may resemble characteristics of myotonic dystrophy, and may lead to an erroneous diagnosis of myotonic dystrophy. |
本系統中英文摘要資訊取自各篇刊載內容。