Peterson J W, Bö L, Mörk S, Chang A, Trapp B D
Neurosciences Graduate Studies Program, Ohio State University, Columbus, USA.
Ann Neurol. 2001 Sep;50(3):389-400. doi: 10.1002/ana.1123.
Multiple Sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system that causes motor, sensory, and cognitive deficits. The present study characterized demyelinated lesions in the cerebral cortex of MS patients. One hundred twelve cortical lesions were identified in 110 tissue blocks from 50 MS patients. Three patterns of cortical demyelination were identified: Type I lesions were contiguous with subcortical white matter lesions; Type II lesions were small, confined to the cortex, and often perivascular; Type III lesions extended from the pial surface to cortical layer 3 or 4. Inflammation and neuronal pathology were studied in tissue from 8 and 7 patients, respectively. Compared to white matter lesions, cortical lesions contained 13 times fewer CD3-positive lymphocytes (195 vs 2,596/mm3 of tissue) and 6 times fewer CD68-positive microglia/macrophages (11,948 vs 67,956/mm3 of tissue). Transected neurites (both axons and dendrites) occurred at a density of 4,119/mm3 in active cortical lesions, 1,107/mm3 in chronic active cortical lesions, 25/mm3 in chronic inactive cortical lesions, 8/mm3 in myelinated MS cortex, and 1/mm3 in control cortex. In active and chronic active cortical lesions, activated microglia closely apposed and ensheathed apical dendrites, neurites, and neuronal perikarya. In addition, apoptotic neurons were increased significantly in demyelinated cortex compared to myelinated cortex. These data support the hypothesis that demyelination, axonal transection, dendritic transection, and apoptotic loss of neurons in the cerebral cortex contribute to neurological dysfunction in MS patients.
多发性硬化症（MS）是一种中枢神经系统的炎性脱髓鞘疾病，可导致运动、感觉和认知功能障碍。本研究对MS患者大脑皮层中的脱髓鞘病变进行了特征描述。在来自50例MS患者的110个组织块中识别出112个皮层病变。确定了三种皮层脱髓鞘模式：I型病变与皮层下白质病变相邻；II型病变较小，局限于皮层，且常为血管周围性；III型病变从软脑膜表面延伸至皮层第3或第4层。分别对8例和7例患者的组织进行了炎症和神经元病理学研究。与白质病变相比，皮层病变中CD3阳性淋巴细胞数量少13倍（每立方毫米组织中分别为195个和2596个），CD68阳性小胶质细胞/巨噬细胞数量少6倍（每立方毫米组织中分别为11948个和67956个）。在活跃的皮层病变中，横断的神经突（轴突和树突）密度为每立方毫米4119个，在慢性活跃皮层病变中为每立方毫米1,107个，在慢性非活跃皮层病变中为每立方毫米25个，在有髓鞘的MS皮层中为每立方毫米8个，在对照皮层中为每立方毫米1个。在活跃和慢性活跃皮层病变中，活化的小胶质细胞紧密贴附并包裹顶端树突、神经突和神经元胞体。此外，与有髓鞘的皮层相比，脱髓鞘皮层中的凋亡神经元显著增加。这些数据支持了以下假说：大脑皮层中的脱髓鞘、轴突横断、树突横断以及神经元的凋亡性丢失导致了MS患者的神经功能障碍。
