Borrajero I, Pérez J L, Domínguez C, Chong A, Coro R M, Rodríguez H, Gómez N, Román G C, Navarro-Román L
National Reference Center for Pathologic Anatomy, Laboratory of Pathology, Havana, Cuba.
J Neurol Sci. 1994 Dec 1;127(1):68-76. doi: 10.1016/0022-510x(94)90137-6.
More than 50,000 patients were affected in Cuba during an epidemic outbreak of peripheral neuropathy from January 1992 until September 1993. The disease presented as either a retrobulbar optic neuropathy, a predominantly sensory peripheral neuropathy, a dorsolateral myeloneuropathy, or as mixed forms. The morphological findings in sural nerve biopsies from 34 patients with various forms of the disease are presented here. Frozen, paraffin and semi-thin sections were prepared for light and electron microscopy, immunohistochemistry and morphometric analysis. Every case presented morphological alterations ranging from mild axonal dystrophy (9 cases, or 27%) to moderate and severe axonal damage (25 cases, or 73%). In 6 cases (18%), axonal damage was accompanied by perineural fibrosis and vascular abnormalities. Axonal regeneration was noted in 8 cases (23%) and remyelination in 9 (26%). Morphometric analysis showed a predominant loss of myelinated fibers in 92% of the patients. Quantification of myelinated fiber loss in 11 patients revealed a remarkable decrease in large caliber fibers. Scarce mononuclear cells were observed in 17 cases. No virus-like elements were seen. The morphological features found in this study indicate that, regardless of the clinical presentation, peripheral nerve lesions of the epidemic neuropathy in Cuba correspond to an axonal neuropathy. These lesions are compatible with nutritional, toxic, or metabolic etiologies. An inflammatory etiology would be unusual with these lesions.
1992年1月至1993年9月古巴流行性周围神经病爆发期间,超过50000名患者受到影响。该疾病表现为球后视神经炎、以感觉为主的周围神经病、脊髓背外侧神经病或混合形式。本文介绍了34例患有各种形式该疾病患者的腓肠神经活检的形态学发现。制备了冰冻、石蜡和半薄切片用于光镜和电镜检查、免疫组织化学和形态计量分析。每个病例均呈现出形态学改变,从轻度轴索营养不良(9例,占27%)到中度和重度轴索损伤(25例,占73%)。在6例(18%)中,轴索损伤伴有神经周纤维化和血管异常。8例(23%)出现轴索再生,9例(26%)出现髓鞘再生。形态计量分析显示在92%的患者中主要是有髓纤维丢失。对11例患者的有髓纤维丢失进行定量分析显示大口径纤维显著减少。17例中观察到少量单核细胞。未见病毒样成分。本研究中发现的形态学特征表明,无论临床表现如何,古巴流行性神经病的周围神经病变均为轴索性神经病。这些病变与营养、中毒或代谢病因相符。炎症性病因在这些病变中不常见。