Liberski P P, Buczyński J, Yanagihara R, Mora C, Gibbs C J, Gajdusek C, Cartier L, Verdugo A, Araya F, Castillo L
Laboratory of Central Nervous System Studies, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA.
Ultrastruct Pathol. 1999 May-Jun;23(3):157-62. doi: 10.1080/019131299281653.
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. Previously, we demonstrated peculiar lamellated structures, called "multilamellar bodies" (MLB). In this report, we present the ultrastructural neuropathology of a TSP/HAM case from Chile, with further detailed descriptions of MLB. It is tempting to suggest that MLB may represent specific ultrastructural markers of TSP/HAM. The pathology of the anterior and posterior horns was similar and was comprised of 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 fibers 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. In a few specimens of the anterior and posterior horns of the spinal cord, MLB were observed. These structures consisted of stacks of 30 to 40 electron-dense lamellae, which were interrupted by narrow electron-lucent spaces. All of the lamellae were immersed within an amorphous substance of intermediate density. Neurons of the dorsal root ganglia were basically normal except for increased lipofuscin accumulation. As in the spinal cord, myelinated axons were well preserved, but a few were demyelinated and surrounded by concentric arrays of Schwann cell membranes. Also, axons of the dorsal roots accumulated increased number of neurofilaments. Mast cells and Schwann cells were increased in number, the latter containing abundant pi granules and myelin fragments.
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较好的了解，但仅基于少数偶发病例的检查。此前，我们发现了一种特殊的层状结构，称为“多层小体”（MLB）。在本报告中，我们展示了一例来自智利的TSP/HAM病例的超微结构神经病理学，并对MLB进行了更详细的描述。很有可能MLB可能代表TSP/HAM的特定超微结构标志物。脊髓前后角的病理变化相似，表现为轴突退变，并伴有广泛的星形胶质细胞增生。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中。超微结构上，髓鞘相对保存完好，观察到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。在脊髓前后角的少数标本中，观察到了MLB。这些结构由30至40个电子致密层堆叠而成，被狭窄的电子透亮间隙中断。所有的层都沉浸在中等密度的无定形物质中。背根神经节的神经元基本正常，只是脂褐素积累增加。与脊髓一样，有髓轴突保存完好，但有少数脱髓鞘并被雪旺细胞膜的同心排列所环绕。此外，背根轴突积累了更多的神经丝。肥大细胞和雪旺细胞数量增加，后者含有丰富的π颗粒和髓鞘碎片。
