论文部分内容阅读
目的 总结腓骨肌萎缩症 (HMSNⅠ型和Ⅱ型 )周围神经的病理改变及其特征。 方法 采用单纤维分离、光镜及电镜观察 5例HMSNⅠ型、2例HMSNⅡ型腓肠神经的病理改变。 结果 光镜下HMSNⅠ型患者大直径有髓纤维几乎完全消失 ,HMSNⅡ型部分保留 ,Ⅰ型各例均可见大量洋葱头样结构 ,Ⅱ型则无。电镜观察同样见到Ⅰ型患者有大量洋葱头样结构 ,并有较多膜性结构和雪旺氏细胞胞浆突起成分。Ⅱ型可见髓鞘坏变、塌陷 ,轴索内空泡变性。 结论 HMSNⅠ型的基本病理改变为雪旺氏细胞病变 ,Ⅱ型则为轴索变性。
Objective To summarize the pathological changes and their characteristics of peripheral nerve of Charcot-Marie-Tooth Syndrome (HMSNⅠ and Ⅱ). Methods The pathological changes of 5 cases of HMSN type I and 2 cases of HMSN type II sural nerve were observed by single fiber separation, light microscope and electron microscope. Results The large diameter myelinated fibers of HMSN Ⅰ type patients disappeared almost completely under the light microscope. Some HMSN Ⅱ types were retained, and a large number of onion - like structures were observed in all cases of type Ⅰ. Electron microscopy also found that type I patients have a large number of onion-like structure, and more membranous structure and Schwann cell cytoplasmic protrusions. Type II visible myelin sheath collapse, collapse, axonal degeneration. Conclusion The basic pathological changes of HMSN Ⅰ type are Schwann cell lesions, and type Ⅱ axonal degeneration.