Neurology Department, University Hospital, 4 rue Larrey, F49933 Angers Cedex 9, France.
Muscle Nerve. 2011 Sep;44(3):332-9. doi: 10.1002/mus.22069.
To detail the extent and pattern of axon cytoskeleton alterations in chronic inflammatory demyelinating polyneuropathy (CIDP).
Nerve biopsies from 7 cases of CIDP, including 4 cases with severe fiber loss, were compared with 5 controls by morphometric transmission electron microscopy (TEM).
Despite demyelination of single fibers, myelin ultrastructure was otherwise normal. Contrary to immunolabeling, TEM revealed a decrease in neurofilament (NF) density in every case, although there were pronounced variations among fibers even in the same sample. The NF decrease reached the same extent in large- and small-diameter fibers. It was observed in normally myelinated fibers, suggesting they were demyelinated at a distance from the section. Minimal inter-NF distance increased roughly inversely to NF density. Microtubules increased in 3 cases previously characterized by increased growth-associated protein (GAP-43) immunolabeling.
These data demonstrate the severity and constancy of axonal lesions, and especially of NF, in residual fibers in our cases of CIDP.
详细描述慢性炎症性脱髓鞘性多发性神经病（CIDP）中轴突细胞骨架改变的程度和模式。
通过形态计量透射电子显微镜（TEM）比较了 7 例 CIDP 病例的神经活检，包括 4 例严重纤维丢失病例，与 5 例对照进行比较。
尽管存在单个纤维脱髓鞘，但髓鞘超微结构正常。与免疫标记相反，TEM 显示在每种情况下 NF 密度均降低，尽管即使在同一样本中，纤维之间也存在明显的变化。NF 减少在大直径和小直径纤维中达到相同的程度。它在正常髓鞘纤维中观察到，表明它们在远离切片的部位脱髓鞘。最小的 NF 间距离大致与 NF 密度成反比增加。在以前用生长相关蛋白（GAP-43）免疫标记显示增加的 3 例中，微管增加。
这些数据表明我们的 CIDP 病例中残留纤维中轴突病变，特别是 NF 的严重程度和一致性。
