Gibbels E, Behse F, Klingmüller G, Henke-Lübke U, Haupt W F, Gollmer E
Department of Neurology, University of Cologne, Köln, FRG.
Clin Neuropathol. 1988 May-Jun;7(3):120-30.
Reports on biopsy findings in multifascicular nerves in lepromatous leprosy (LL) are rare and detailed morphometrical data are not available. In a case of early LL with normal electrodiagnostic findings in sural nerve, the present study revealed marked segmental de- and remyelination concomitant with the sequelae of considerable Wallerian degeneration of preferentially small myelinated fibers (MF) in spite of a normal number/nerve and density/mm2. Segmental de- and remyelination of several consecutive internodes in teased fibers suggests continuous bacterial spread via Schwann cells. In 2 more advanced LL-cases, nervous parenchyme was severely reduced, in a borderline lepromatous (BL) case obviously in part caused by cell infiltrates and granulomata. Distinct fascicle differences in MF-involvement were demonstrated by coefficients of variation of MF/mm2 and teased fiber preparations in LL, consistent with the hypothesis of initial focal spread of bacteria. Numbers and densities of endoneurial vessels were increased only in the later stages of LL. Enlargement of endoneurial area, due to different factors, was encountered except for the most severe LL-case with extensive endoneurial collagenization. Morphometric results were compared with those of other neuropathies. Intact and degenerating bacteria mostly in phagosomes of Schwann cells associated with unmyelinated axons and in macrophages were seen only in the early LL- and the BL-case. Sparse mononuclear cell infiltrates and small focal necrosis, present even in LL-cases, underline the complex pathogenesis of nerve fiber involvement.
关于瘤型麻风(LL)多束神经活检结果的报道很少,且缺乏详细的形态学测量数据。在一例腓肠神经电诊断结果正常的早期LL病例中,本研究发现尽管每根神经的数量和每平方毫米的密度正常,但仍有明显的节段性脱髓鞘和髓鞘再生,同时伴有优先累及的小髓鞘纤维(MF)严重华勒氏变性的后遗症。 teased纤维中几个连续节段的节段性脱髓鞘和髓鞘再生提示细菌通过施万细胞持续扩散。在另外2例更晚期的LL病例中,神经实质严重减少,在1例界线类偏瘤型(BL)病例中,明显部分是由细胞浸润和肉芽肿引起的。通过LL中MF/mm2的变异系数和teased纤维制备,证明了MF受累存在明显的束差异,这与细菌最初局灶性扩散的假设一致。仅在LL后期,神经内膜血管的数量和密度增加。除了最严重的伴有广泛神经内膜胶原化的LL病例外,由于不同因素,神经内膜面积增大。将形态学测量结果与其他神经病变的结果进行了比较。完整和退化的细菌主要存在于与无髓鞘轴突相关的施万细胞吞噬体和巨噬细胞中,仅在早期LL病例和BL病例中可见。即使在LL病例中也存在的稀疏单核细胞浸润和小灶性坏死强调了神经纤维受累的复杂发病机制。