Chimelli L, Freitas M, Nascimento O
Department of Pathology, School of Medicine, Fluminense Federal University, Niterói, RJ, Brazil.
J Neurol. 1997 May;244(5):318-23. doi: 10.1007/s004150050094.
Nerve biopsy specimens from 53 patients with leprosy and neuropathy were taken from the sural, the dorsal branch of the ulnar, or the superficial radial nerves and processed for light and electron microscopy. There was inflammation in 40 cases (75%), 7 with a granulomatous reaction, various stages of fibrosis in 35 (66%), and endoneurial vascular neoformation in 7. In two cases, small focal infarcts were associated with marked endoneurial inflammation compressing the vessels, in addition to endoneurial lymphocytic vasculitis. Most had an axonal neuropathy of varying degree, some with total fibre loss, others with predominant small myelinated and unmyelinated fibre loss. Signs of demyelination and remyelination were the main findings in 9 cases (17%). Bacilli were present in endothelial, perineurial, Schwann cells and in macrophages. On two occasions, they lost their alcohol acid resistance, were suspected in semithin sections, and confirmed ultrastructurally. The biopsy was decisive for the diagnosis of leprosy in 15 cases (28%), most without skin lesions. We evaluated the effectiveness of the treatment in 20 (37.7%), 12 without and 8 with bacilli, despite negativity in the skin. The diagnosis of leprosy based on skin lesions was confirmed with the nerve biopsy in 9 cases, 6 had an inflammatory neuropathy suggestive of leprosy in the absence of bacilli, and 3 had nonspecific changes in the sural nerve since the neuropathy was in the upper limbs. We conclude that nerve biopsy is indicated for the diagnosis of leprosy in cases without clinically visible skin lesions and to evaluate the effectiveness of the treatment. In these cases the ultrastructural studies are important for recognition of the bacilli. Vascular lesions may play an important role in the progression of the nerve damage, including the occurrence of focal nerve infarcts which, to our knowledge, have not been previously reported in association with leprosy.
取自53例麻风病伴神经病变患者的腓肠神经、尺神经背支或桡神经浅支的神经活检标本,进行光镜和电镜检查。40例(75%)有炎症,7例有肉芽肿反应,35例(66%)有不同阶段的纤维化,7例有神经内膜血管新生。2例除神经内膜淋巴细胞性血管炎外,小灶性梗死与压迫血管的明显神经内膜炎症相关。多数患者有不同程度的轴索性神经病变,部分患者神经纤维完全丧失,另一些患者主要是小的有髓和无髓神经纤维丧失。9例(17%)的主要发现为脱髓鞘和再髓鞘化迹象。内皮细胞、神经束膜细胞、施万细胞和巨噬细胞内均有杆菌。有两次,杆菌失去抗酸性酒精能力,在半薄切片中被怀疑,超微结构检查得以证实。活检对15例(28%)麻风病的诊断起决定性作用,多数患者无皮肤损害。我们评估了20例(37.7%)患者的治疗效果,其中12例无杆菌,8例有杆菌,尽管皮肤检查为阴性。9例经神经活检证实基于皮肤损害诊断的麻风病,6例在无杆菌情况下有提示麻风病的炎性神经病变,3例因神经病变在上肢,腓肠神经有非特异性改变。我们得出结论,对于无临床可见皮肤损害的病例,神经活检有助于麻风病的诊断及评估治疗效果。在这些病例中,超微结构研究对于杆菌的识别很重要。血管病变可能在神经损害的进展中起重要作用,包括局灶性神经梗死的发生,据我们所知以前尚未报道其与麻风病相关。