【摘要】 目的:皮质基底节综合征(corticobasal syndrome, CBS)是一种缓慢进展的神经退行性疾病,特征为不对称的皮质萎缩和锥体外系症状。现将我院收治的2例进行报道,探讨皮质基底节变性综合征的临床表现、神经心理学、影像学和病理特征。方法:对2例临床诊断为皮质基底节综合征患者的资料进行回顾分析,并进行文献综述。结果:2例患者均有不对称性肢体肌张力增高、肢体失用、记忆力和视空间损害。均合并有进行性非流理性失语,病例1伴有意向性震颤。神心理量表提示2例患者均有认知损害,视空间和记忆力下降尤为显著。头颅磁共振均有明显的脑萎缩,以额顶叶显著。2例患者脑脊液病理符合阿尔茨海默病病理改变。结论:不对称性肌张力增高、肢体失用以及视空间障碍是皮质基底节综合征较为常见的临床表现,详细的神经心理学量表、影像学及脑脊液病理检测可提高疾病的诊断以及指导治疗。更多还原

【Abstract】 Objective Corticobasal syndrome(CBS) is a progressive neurodegenerative disease that characterized by asymmetrical cortical atrophy and parkinsonism. CBS regarded as a rare neurodegenerative disorder, and is difficult to diagnose by clinician. The study is aim to investigate clinical feature, neuropsychological, imaging and pathology of corticobasal syndrome. Methods We admitted two patients with clinical diagnosis of CBS in our hospital, and analyzed the feature of clinical symptoms, neuropsychological, imaging and pathology. Results Two patients had asymmetry limb rigidity, limb apraxia, memory impairment and visuospatial disturbance. All patents presented with progressive non-fluent aphasia, case 1 was complicated by tremor. The neuropsychological assessment suggests that both patients have cognitive impairment, especially in visuospatial and memory. Brain MRI showed that all the patients had dominantly frontoparietal cortical atrophy. Cerebrospinal fluid test revealed 2 patients with underlying Alzheimer pathology. Conclusion Asymmetry limb rigidity, limb apraxia, and visuospatial disturbance are the common clinical feature of corticobasal syndrome. Detailed neuropsychological test, MRI and pathological examination are helpful to the diagnosis of CBS.更多还原