Said G
Rev Neurol (Paris). 1976 Jul;132(7):467-80.
This report describes pathological changes of myelin, cells and axons of teased nerve fibers from three cases (two having a family history) of primary hypertrophic neuritis with onion bulb formations seen in aptic and electron microscopy. Segmental demyelination and subsequent remyelination occurred in most myelinated fibers isolated. Besides this classical pattern, in some fibers large pseudo-wallerian ovoids of myelin with preservation of the axon, demonstrated by silver counterstaining, were found. This pseudo-wallerian degeneration is thus a primary demyelination and axonal degeneration remains a rare finding. A quantitative evaluation of the different patterns of myelin degeneration has been established. The study of cellular proliferation has shown clustering of nuclei scattered along the fiber. In some fibers the changes of the myelin sheath are encountered without Schwann-cell accummulation, while in others cellular proliferation is prominent without obvious myelin abnormalities. Cellular proliferation has also been seen in unmyelinated fibers (Remak fibers). In some neural tubes devoid of axis cylinders striking Schwann-cell accumulation remains, giving may in some places to fusiform enlargement of the "Schwann-cell tube". These "Schwann-cell tubes" appear as one of the late stage changes occuring in hypertrophic neuritis.
本报告描述了3例（其中2例有家族病史）原发性肥厚性神经炎的 teased 神经纤维的髓鞘、细胞和轴突的病理变化，这些变化在光学显微镜和电子显微镜下可见洋葱球形成。在大多数分离出的有髓纤维中发生了节段性脱髓鞘及随后的再髓鞘化。除了这种经典模式外，在一些纤维中还发现了经银复染显示的、轴突保存的大的髓鞘假华勒氏卵圆形结构。因此，这种假华勒氏变性是一种原发性脱髓鞘，而轴突变性仍然是罕见的发现。已经建立了对髓鞘变性不同模式的定量评估。细胞增殖研究显示细胞核沿纤维呈簇状分布。在一些纤维中，髓鞘的变化在没有施万细胞聚集的情况下出现，而在另一些纤维中，细胞增殖显著但没有明显的髓鞘异常。在无髓纤维（雷马克纤维）中也可见细胞增殖。在一些没有轴突圆柱体的神经管中，施万细胞的显著聚集仍然存在，在某些地方可导致“施万细胞管”呈梭形扩大。这些“施万细胞管”似乎是肥厚性神经炎发生的晚期变化之一。
