Li Wenzhu, Zheng Yiming, Zhang Wei, Wang Zhaoxia, Xiao Jiangxi, Yuan Yun
Department of Neurology, Peking University First Hospital, China.
Department of Radiology, Peking University First Hospital, China.
Neuromuscul Disord. 2015 May;25(5):375-80. doi: 10.1016/j.nmd.2015.01.003. Epub 2015 Jan 19.
The purpose of this study was to assess the progression and variation of fatty infiltration of the thigh muscles of Duchenne muscular dystrophy patients. Muscle magnetic resonance imaging was used to measure the degree of fatty infiltration of the thigh muscles of 171 boys with Duchenne muscular dystrophy (mean age, 6.09 ± 2.30 years). Fatty infiltration was assigned using a modified Mercuri's scale 0-5 (normal-severe). The gluteus maximus and adductor magnus were affected in patients less than two years old, followed by the biceps femoris. Quadriceps and semimembranosus were first affected at the age of five to six years; the sartorius, gracilis and adductor longus remained apparently unaffected until seven years of age. Fatty infiltration of all the thigh muscles developed rapidly after seven years of age. The standard deviation of the fatty infiltration scores ranged from 2.41 to 4.87 before five years old, and from 6.84 to 11.66 between six and ten years old. This study provides evidence of highly variable degrees of fatty infiltration in children of different ages with Duchenne muscular dystrophy, and indicates that fatty infiltration progresses more quickly after seven years of age. These findings may be beneficial for the selection of therapeutic regimens and the analysis of future clinical trials.
本研究的目的是评估杜氏肌营养不良症患者大腿肌肉脂肪浸润的进展和变化。采用肌肉磁共振成像测量171例杜氏肌营养不良症男孩（平均年龄6.09±2.30岁）大腿肌肉的脂肪浸润程度。使用改良的Mercuri量表0-5（正常-严重）对脂肪浸润进行分级。臀大肌和大收肌在2岁以下的患者中受到影响，其次是股二头肌。股四头肌和半膜肌在5至6岁时首次受到影响；缝匠肌、股薄肌和长收肌在7岁之前明显未受影响。7岁以后，所有大腿肌肉的脂肪浸润迅速发展。5岁前脂肪浸润评分的标准差为2.41至4.87，6至10岁之间为6.84至11.66。本研究提供了不同年龄的杜氏肌营养不良症患儿脂肪浸润程度高度可变的证据，并表明7岁以后脂肪浸润进展更快。这些发现可能有助于治疗方案的选择和未来临床试验的分析。
