Buczyński J, Yanagihara R, Mora C, Cartier L, Verdugo A, Araya F, Castillo L, Gibbs C J, Gajdusek C D, Rogers-Johnson P, Liberski P P
Laboratory of Electron Microscopy and Neuropathology, Department of Molecular Biology, Medical Academy, Lódź, Poland.
Folia Neuropathol. 2001;39(4):265-9.
Human T-cell lymphotropic virus type I (HTLV-I) is the cause of endemic tropical spastic paraparesis (TSP) or HTLV-I-associated myelopathy (HAM). Because TSP/HAM is not a fatal disease, the neuropathology of this disease, albeit relatively well understood, is based on the examination of just a few incidental cases. We summarise our experience with the neuropathology of tropical spastic paraparesis/HTLV-I associated myelopathy (TSP/HAM). We studied three cases of TSP/HAM from different parts of the world. We demonstrated peculiar lamellated structures, called "multilamellar bodies" (MLB). It is tempting to suggest that MLB may represent specific ultrastructural markers of TSP/HAM. The pathology of the anteriorand posterior horns was similar and comprised axonal degeneration, accompanied by extensive astrocytic gliosis. Lymphocytic infiltration, particularly observed as "cuffs" around blood vessels, was scattered among other cellular elements. Ultrastructurally, myelin sheaths were relatively well preserved, and some demyelinated but not remyelinated fibres were observed. Moreover, axons with abnormal accumulations of neurofilaments, suggestive of axonal degeneration, were detected. Several axons contained Hirano bodies. In many samples glial processes replaced most of the remaining neuropil.
人类嗜T细胞病毒I型(HTLV-I)是地方性热带痉挛性截瘫(TSP)或HTLV-I相关脊髓病(HAM)的病因。由于TSP/HAM并非致命疾病,尽管对该疾病的神经病理学已有相对深入的了解,但这些认识仅基于少数偶发病例的检查。我们总结了我们在热带痉挛性截瘫/HTLV-I相关脊髓病(TSP/HAM)神经病理学方面的经验。我们研究了来自世界不同地区的3例TSP/HAM病例。我们发现了特殊的层状结构,称为“多层小体”(MLB)。很有可能认为MLB可能代表TSP/HAM的特异性超微结构标志物。前角和后角的病理变化相似,包括轴突退变,并伴有广泛的星形细胞胶质增生。淋巴细胞浸润,尤其是在血管周围呈“套袖状”,散在于其他细胞成分之间。超微结构上,髓鞘相对保存完好,可见一些脱髓鞘但未再髓鞘化的纤维。此外,检测到神经丝异常聚集的轴突,提示轴突退变。一些轴突含有 Hirano小体。在许多样本中,胶质细胞突起取代了大部分剩余的神经毡。