以電腦斷層評估顏面肩胛上腕型肌失養症之肌肉影響

Muscle Involvement in Facioscapulohumeral Muscular Dystrophy Assessed by Computed Tomography

王建華(Chien-Hua Wang)

指導教授 ： 鐘育志

高雄醫學大學/醫學院/醫學研究所/碩士(2010年)

摘要

中文摘要
本研究的目的是利用電腦斷層(computed tomography, CT)來評估24位顏面肩胛上腕型肌失養症(facioscapulohumeral muscular dystrophy, FSHD)患者之肌肉影像的影響程度。本論文收集在本校附設醫院24位FSHD患者的肌肉CT影像，所有的肌肉影像評估是根據四個半定量等級 (four-point semi-quantitative visual scale)。CT肌肉影像評估的結果與D4Z4基因片斷大小 ( fragment size of D4Z4)、發病年齡、患病時間(disease duration)、臨床嚴重度 (clinical severity score)、血清肌酸激酶(serum creatine kinase)及肌肉力量去作相關性的比較。上肢影響最嚴重的肌肉是斜方肌(trapezium)；下肢最嚴重的肌肉則是膕肌(hamstring muscles)，包含股二頭肌(biceps femoris)、半腱肌(semitendinosus)、半膜肌(semimembranosus)。上肢肌肉則是棘上肌(supraspinatus)、肩胛下肌(subscapularis)是最不受影響的肌肉；下肢的股四頭肌(quadriceps femoris)，包含股外側肌(vastus lateralis)、股內側肌 (vastus medialis)、股中間肌(vastus intermedius)、股直肌(rectus femoris) 和比目魚肌(soleus)與腓骨肌(fibularis)是最不受影響的肌肉。脊椎旁肌肉(paraspinal muscles)異常常見於年紀較大的FSHD患者。CT顯示上肢肌肉兩邊不對稱的比例(27%)明顯大於下肢肌肉(18%)。利用徒手肌力測試(manual muscle test, 簡稱MMT)顯示上肢與下肢所檢查的肌力測量，兩邊不對稱的比例分別是21%與14%。上下肢的CT肌肉影像的評估結果，顯示與其臨床嚴重度有正相關(上肢：r=0.83, p<0.0001;下肢：r=0.76, p<0.0001)，與D4Z4 fragment size有負相關(上肢：r=-0.48, p<0.05;下肢：r=-0.67, p<0.005)，但是和發病年齡沒有相關。只有在上肢肌肉的CT影像評估結果，和患病時間長短有正相關(r=0.49, p<0.05)。在下肢肌肉的CT影像評估結果，和血清肌酸激酶有正相關(r=0.6, p=0.005)。CT的肌肉影像可以揭開執行髖關節屈曲(hip flexion)與膝關節伸長(knee extension)、踝關節的背側屈曲(dorsiflexion)等相關肌肉之病變，但是這些動作卻無法單純用MMT檢測到肌力的降低。因此，CT肌肉影像評估可以增加兩側不對稱肌肉影響的偵測率，與及早發現FSHD特殊肌肉好發處，以提供臨床醫師診斷及鑑別診斷FSHD之重要工具。

關鍵字

顏面肩胛上腕型肌失養症 ； 肌肉影響

並列摘要

Abstract
The objective of this study aims to evaluate the degree of involvement of skeletal muscles in 24 unselected patients with facioscapulohumeral muscular dystrophy (FSHD) by computed tomography (CT). Twenty-four FSHD patients regularly followed at Kaohsiung Medical University Hospital were enrolled in this study. All of the evaluations and grading of the muscle imaging which detected by CT, were based on a four-point semi-quantitative visual scale. Muscle gradings were correlated with fragment size of D4Z4, age of onset, disease duration, clinical severity scores, serum creatine kinase values and muscle power. Mostly affected muscles were the trapezium in the upper limbs, followed by the hamstrings (including semitendinosus, semimembranosus and biceps femoris) in the lower limbs. In the upper limbs, the supraspinatus and subscapularis were the least unaffected ; the quadriceps femoris (including vastus lateralis, vastus medialis, vastus intermedius and rectus femoris), soleus and peroneal muscles showed the least unaffected muscles in the lower limbs. The paraspinal muscles were often seen in old FSHD patients. Asymmetric involvement was more evident (27%) in the investigated muscles in the upper limbs than those (18%) in the lower limbs on CT. Manual muscle testing (MMT) revealed asymmetric strength in investigated joint movements of the upper (21%) and lower limbs (14%). CT findings in muscles of the four extremities showed a strong correlation with clinical severity scores (r=0.83, p<0.0001) in the upper limbs and (r=0.76, p<0.0001) in the lower limbs, and a negative correlation with D4Z4 fragment sizes ( r= -0.48, p<0.05) in the upper limbs and (r=-0.67, p<0.005) in the lower limbs, but not with age of onset. Only CT findings in muscle of the upper limbs tended to be correlate with disease duration (r = 0.49; p < 0.05). CT findings in muscles of the lower limbs were correlated with serum creatine kinase (r=0.60, p=0.005). It was evident that CT disclosed muscles involved in hip flexion, knee extension and ankle dorsal flexion that could not be detected by MMT. Furthermore, based on increased asymmetry that was not detected on MMT but early detection of muscle involvement by CT in FSHD, it suggests that muscle CT may play a crucial role in the process of diagnosis and differential diagnosis of FSHD.

並列關鍵字

Facioscapulohumeral Muscular Dystrophy ； Muscle Involvement

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