目的　阐述皮质基底节变性的临床和病理特征。方法　报告 2例皮质基底节变性的临床资料和病理检查结果。结果　 2例病人早期表现为单侧肢体运动障碍如肌张力增高 ,跌倒发作 ,病程中出现四肢强直少动 ,伴肌阵挛和不同程度的皮层认知功能障碍 ;影像学表现为额顶叶不对称性萎缩或脑代谢减低。大体见普遍脑萎缩 ,但以额、顶叶皮层为著 ,镜下检查见皮层气球样神经元 ,基底节和黑质神经细胞变性脱失。Gallyas Braak银染色和Tau免疫组织化学染色显示基底节、黑质等神经元胞质内Tau阳性球形包涵体 ,额叶、顶叶皮层及基底节大量Tau阳性簇状星形胶质细胞和星形胶质细胞斑。结论　老年人慢性进展性不对称肢体运动障碍伴痴呆时 ,临床应考虑到皮质基底节变性。如病理上发现特异性神经元及星形胶质细胞改变 ,则有助于本病的确诊 Objective To describe the clinical and pathological features of cortical basal ganglia degeneration. Methods Two cases of cortical basal ganglia degeneration clinical data and pathological findings were reported. Results Early manifestation of unilateral limb dyskinesia such as increased muscle tension and fall occurred in 2 patients. During the course of the disease, there appeared tetanic atretic limbs with myoclonus and different degrees of cortical cognitive dysfunction. Symmetry atrophy or decreased brain metabolism. Generally see the general brain atrophy, but the amount of the parietal cortex as a mirror examination showed cortical balloon-like neurons, basal ganglia and degeneration of substantia nigra neurons. Gallyas Braak silver staining and Tau immunohistochemical staining showed that Tau-positive spherical inclusions in neuronal cytoplasm, such as basal ganglia and substantia nigra, a large number of Tau-positive clustered astrocytes in the frontal lobe, parietal cortex and basal ganglia, Glial cell spots. Conclusion The elderly patients with chronic progressive asymmetric limb movement disorder with dementia should be considered in clinical cortical basal ganglia degeneration. If the pathological changes found in specific neurons and astrocytes, then help diagnose the disease