DTI评估Duchenne型肌营养不良症严重程度的初步研究

Preliminary study of DTI in assessing disease severity in children with Duchenne muscular dystrophy

目的:探讨DTI评估Duchenne肌营养不良症(DMD)患儿病情严重程度的临床价值。方法:28例DMD患儿均行大腿肌肉常规MRI和DTI检查,测量右侧大腿14块肌肉(臀大肌、阔筋膜张肌、股外侧肌、股中间肌、股内侧肌、股直肌、缝匠肌、长收肌、大收肌、股薄肌、半膜肌、半腱肌、股二头肌长头、股二头肌短头)的各向异性分数(FA)值、ADC值。根据运动功能评估量表(MFM)评价患儿运动功能,并分析各肌肉FA值、ADC值和14块肌肉平均FA值、ADC值与MFM总评分的相关性。结果:除缝匠肌、股薄肌、半膜肌及半腱肌外,其余肌肉FA值与MFM总评分均呈负相关(均P<0.05),ADC值与MFM总评分均呈正相关(均P<0.05);FA值以臀大肌相关性最高(r=-0.587),ADC值以股外侧肌相关性最高(r=0.470)。14块肌肉平均FA值与MFM总评分呈负相关(r=-0.539,P<0.05),平均ADC值与MFM总评分呈正相关(r=0.478,P<0.05)。结论:DTI可用于量化和客观分析DMD患儿的临床严重程度。臀大肌FA值与MFM总评分相关性最高,提示臀大肌FA值测量可能是反映DMD患儿运动功能的理想指标。

Objective:To explore the clinical value of DTI in evaluating the disease severity in children with Duchenne muscular dystrophy(DMD).Methods:Twenty-eight children with DMD underwent conventional MRI and DTI of thigh muscles.The FA value and ADC value of 14 right thigh muscles(gluteus maximus,tensor fascia lata,lateral femoris,middle femoris,medial femoris,rectus femoris,sartorius,adductor longus,adductor major,gracilis,semimembranous,semitendinosus,biceps femoris long head and short head)were measured.The motor function of children was evaluated by using Motor Function Measure(MFM)scale.The correlation of FA and ADC value of each muscle,the average FA and ADC value of 14 muscles with the total MFM score was analyzed.Results:Except sartorius,gracilis,semimembranous and semitendinosus,FA values of the other muscles were negatively correlated with the total MFM score(all P<0.05),and ADC values were positively correlated with the total MFM score(all P<0.05).FA value of gluteus maximus had the highest correlation with the total MFM score(r=-0.587),and ADC value of lateral femoris had the highest correlation(r=0.470).The average FA value of 14 muscles was negatively correlated with the total MFM score(r=-0.539,P<0.05),and the average ADC value was positively correlated with the total MFM score(r=0.478,P<0.05).Conclusions:DTI can be used to quantify and objectively analyze the clinical severity of children with DMD.FA value of gluteus maximus has the highest correlation with the total MFM score,suggesting that FA value of gluteus maximus may be an ideal index to reflect the motor function of children with DMD.