Di Trapani G, David P, La Cara A, Tonali P, Laurienzo P
Adv Exp Med Biol. 1987;209:111-9. doi: 10.1007/978-1-4684-5302-7_18.
The aim of this work was to study the neuropathological picture of the sural nerve in the pseudopolineuropathic form of A.L.S. Five patients were considered: in all cases the clinical and electromyographic follow-up excluded other diseases. EMG-studies were repeatedly performed: they showed the progressive evolution of the spinal anterior horn cell pathology from lower spinal to cervical levels. The sural nerve was processed according to the procedure used in our laboratory for light and ultrastructural examination. Quantitative analysis of myelinated fiber density was carried out on photographic enlargement and reported on histograms. The light and ultrastructural examination showed a severe loss of myelinated fibers; the decrease affected all types of fibers, but predominated in the largest ones. In the axons there was an increase of mitochondria, dilation of the small vesicles and increase in the number of neurofilaments. It is our opinion that all histopathologic data obtained in the sural nerve biopsy in this form of A.L.S. reveal a clear involvement of the sensory neurons of the spinal ganglia and the results can be useful for the study of precocius lesions in the A.L.S.
这项工作的目的是研究肌萎缩侧索硬化症假性多神经病形式中腓肠神经的神经病理学情况。研究了5例患者：所有病例的临床和肌电图随访均排除了其他疾病。反复进行肌电图研究：结果显示脊髓前角细胞病变从脊髓下部向颈部逐渐发展。按照我们实验室用于光镜和超微结构检查的程序处理腓肠神经。在放大的照片上对有髓纤维密度进行定量分析，并绘制直方图报告结果。光镜和超微结构检查显示有髓纤维严重缺失；这种减少影响了所有类型的纤维，但以最大的纤维为主。轴突中线粒体增加、小泡扩张且神经丝数量增多。我们认为，在这种形式的肌萎缩侧索硬化症中，腓肠神经活检获得的所有组织病理学数据都显示脊髓神经节感觉神经元明显受累，这些结果可能有助于研究肌萎缩侧索硬化症的早期病变。
