Neuromuscular Centre Nijmegen, Department of Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
Neuromuscul Disord. 2012 Jun;22(6):492-9. doi: 10.1016/j.nmd.2012.01.006. Epub 2012 Mar 11.
This study determines the presence and extent of muscle changes in 31 myotonic dystrophy type 2 (DM2) patients detected by muscle ultrasound. Results were compared to 31 adult-onset myotonic dystrophy type 1 patients (DM1) and healthy controls. Furthermore, we tested the hypothesis that structural muscle changes correlate with age, quantitative muscle force and serum creatine kinase in both disorders. In DM2 all seven examined muscles (right masseter muscle, right and left biceps brachii, right and left forearm flexors, right rectus femoris, and left tibialis anterior muscle) showed increased mean echo intensities (p ≤ 0.001). Atrophy of the masseter muscle and rectus femoris were both found in 23% of DM2 patients. Muscle thickness was significantly more decreased in the elbow flexors in DM2 compared to DM1. Echo intensity sum score correlated positively with age in DM2 (r=0.57, p=0.001) and negatively with muscle force (r=0.36, p=0.048). We conclude that all tested muscles are affected and structurally abnormal in DM2 patients. Proximal arm muscles are more affected in DM2 compared to DM1, which corresponds to clinical findings.
本研究通过肌肉超声检测到 31 例肌强直性营养不良 2 型（DM2）患者的肌肉变化情况，并将结果与 31 例成年发病的肌强直性营养不良 1 型（DM1）患者和健康对照组进行比较。此外，我们还检验了以下假设，即两种疾病中结构肌肉变化与年龄、定量肌肉力量和血清肌酸激酶呈正相关。在 DM2 中，所有 7 块受检肌肉（右侧咬肌、右侧和左侧肱二头肌、右侧和左侧前臂屈肌、右侧股直肌和左侧胫骨前肌）的平均回声强度均增加（p≤0.001）。23%的 DM2 患者出现了咬肌和股直肌萎缩。与 DM1 相比，DM2 患者的肘屈肌厚度明显减小。DM2 中的回声强度总和与年龄呈正相关（r=0.57，p=0.001），与肌肉力量呈负相关（r=-0.36，p=0.048）。我们的结论是，DM2 患者的所有受检肌肉均受到影响且结构异常。与 DM1 相比，DM2 中近端臂肌的受累更为严重，这与临床发现相符。
