Myotonia Dystrophica in a Victim of Traumatic Brain Injury: A Case Report

萎縮性肌強直症合併頭部外傷：病例報告

謝正宜(Jeng-Yi Shieh) ； 張權維(Chein-Wei Chang) ； 連倚南(I-Nan Lien)

《中華民國復健醫學會雜誌》 21期 (1993/12) Pp. 155-160

https://doi.org/10.6315/JRMA.199312.0010

摘要

肌強直是一種肌肉持續收縮的狀態，而萎縮性肌強直是肌強直疾病中較常見的一種。它在1909年首次由Steinert加以詳述，所以又稱為Steinert氏病。它的特色是除了肌強直外，有全身各系統的症狀。包括肌肉萎縮，禿頭，眼險下垂，白內障，聽力障礙，心律不整，性腺萎縮，代謝障礙及智障等。
本篇病例報告描述一個33歲因頭部外傷而轉入復健病房的男性萎縮性肌強直症的病人。鄰床上有禿頭，眼瞼下垂，咬肌萎縮，右小腿肌肉萎縮，聽力障礙，心律不整，性腺萎縮及智障。肌電圖檢查呈現典型肌強直連續肌電波，類似轟炸機俯衝的聲音。肌肉病理組織學檢查發現肌細胞內有明顯內核(internal nuclei)，漿團(sarcolemmal mass)，及環狀纖維(ring fiber)。病人因腦傷雖導致右側肢體麻痺，但無明顯肌痙攣性(spasticity)，其原肌強直性也無明顯改變。

並列摘要

Myotonia dystrophica is one of the common adult-onset muscular dystrophies. It is characterized by its multisystemic involvement as compared with other myotonic disorders. We report here a 33-year-old man with myotonia dystrophica who was diagnosed after transfer to the rehabilitation department for the sequelae of traumatic brain injury, The clinical, electrophysiological and histopathological findings supported the existence of myotonia dystrophica. The presentation of myotonia did not change in association with his central paralysis.

並列關鍵字

myotonia dystrophica ； traumatic brain injury