Comparative morphometric evaluation of peripheral nerves and muscle fibers in myotonic dystrophy
Comparative morphometric evaluation of peripheral nerves and muscle fibers in myotonic dystrophy
复制标题
强直性肌营养不良周围神经和肌纤维的比较形态测量评估
DOI：
10.1007/pl00007404
复制
发表时间：
2000
影响因子：
12.7
通讯作者：
J. Schröder
中科院分区：
文献类型：
--
作者：
J. ;J. Schröder
我们使用计算机辅助的形态测量装置比较了肌强直性营养不良（MD）患者的周围神经纤维和肌肉纤维。在17例MD病例中，14例（82%）的腓肠神经显示髓鞘面积（MSA）与神经内膜面积不同程度的缩小。其中，8例（47%）表现为轻度MSA复位，5例（29.4%）表现为中度复位，1例（6%）表现为重度复位。与对照神经相比，MD中有髓神经纤维的数量没有明显减少，这是由于小的再生神经纤维成团。17例患者中有6例肌纤维平均直径小于40 μm。在这6个严重影响的病例中，¶5显示了MSA的相当大的减少。其他在肌纤维直径方面看起来正常的病例显示不同程度的MSA缩小。因此，周围神经和肌肉之间的变化的严重程度通常存在但并非总是存在形态计量学相关性。周围神经病变的严重程度似乎在很大程度上取决于患者的年龄、疾病的阶段和进展的时间。电子显微镜检查腓肠神经显示明显的病理改变，但非特异性。
We compared peripheral nerve fibers and muscle fibers in myotonic dystrophy (MD) using a computer-assisted device for morphometry. In the 17 cases with MD studied, the sural nerves of 14 cases (82%) showed various degrees of reduction of the myelin sheath area (MSA) per endoneurial area. Of these, 8 cases (47%) presented with a mild reduction of the MSA, 5 cases (29.4%) with moderate reduction, and one case (6%) with severe reduction. The number of myelinated nerve fibers was not significantly reduced in MD when compared with control nerves, due to clusters of small regenerated nerve fibers. The mean diameter of the muscle fibers in 6 of the 17 cases was less than 40 μm. Of these 6 severely affected cases, ¶5 revealed a considerable reduction of the MSA. Other cases, which appeared to be normal in respect to the diameter of muscle fibers, showed various degrees of reduction of the MSA. Thus, there is usually, but not always a morphometric correlation of the severity of changes between peripheral nerves and muscle. The severity of the peripheral neuropathy appears to depend largely on the patient’s age, the stage of the disorder, and the time of progression. Electron microscopic examination of sural nerves showed significant, though non-specific pathological changes.