脑性瘫痪周围神经及骨骼肌亚显微病变研究

Ultrastuctural study on the peripheral nerve and skeletal muscle of patients with spastic cerebral palsy

目的 探讨痉挛性脑瘫周围神经及骨骼肌的病理改变特点。 方法 取材于 2 5例临床确诊为痉挛性脑瘫患者的周围神经及骨骼肌组织 ,进行术中大体观察、光镜和电镜观察。 结果 大体观察基本正常。光镜下 ,骨骼肌纤维有集簇性变性、结缔组织增生 ,观察到的肌梭结构尚好。电镜下 ,周围神经广泛脱髓鞘病变 ,Schwann细胞病变先于并严重于轴索 ;有髓纤维病变重于无髓纤维 ,神经束膜内微血管周有淋巴细胞浸润 ;骨骼肌纤维线粒体多 ,三联管结构少、发育差 ,病变肌纤维呈细胞水肿和肌原纤维破坏表现 ;周围神经及骨骼肌纤维均未见再生迹象。 结论 痉挛性脑瘫的周围神经呈原发性脱髓鞘病变。骨骼肌存在着继发性病损 ,Ⅰ型肌纤维的超微结构表现突出 ;周围神经及骨骼肌再生能力差。

Objective [WT5”BZ]To explore pathological features of the peripheral nerve and skeletal muscle of patients with spastic cerebral palsy. [WT5”HZ]Methods [WT5”BZ]The peripheral nerve and skeletal muscle of 25 patients with spastic cerebral palsy were observed by gross examination during operation ,and under microscope and electron microscope. [WT5”HZ]Results [WT5”BZ]Gross examination showed that peripheral nerve and skeletal muscle was normal during operation. Microscope showed that some skeletal muscle fibers degenerated, and the connective tissue proliferated. electronmicroscopically the damage of demyelination was extensive on the peripheral nerve; the pathological changes of Schwann′s cell were sooner and more severe than axons; the pathological changes of myelinated nerve fiber were more obvious than the nonmyelinated nerve fiber; the lymphocyte were found around the microvasculature within the perineurium; the mitochondrion of skeletal muscle fiber increased in number, the sarcoplasmic reticulum and transverse tubule decreased and developed badly, the degenerative muscle fiber showed cell edema or the pathological changes of myofibril. All the peripheral nerve and skeletal muscle fiber affected had no repair and regeneration. [WT5”HZ]Conclusions[WT5”BZ] The demyelination of the peripheral nerve of patients with spastic cerebral palsy is primary, and the skeletal muscle of patients with spastic cerebral palsy is secondary pathological change. The activity of nerve and muscle regeneration is bad. [WT5”HZ]