皮质基底节综合征患者临床表现影像学及病理学特点分析

摘要

Objective To investigate the clinical,neuroimaging and pathological features of patients with corticobasal syndrome (CBS).Methods Three CBS patients underwent neuropsychological assessment,MRI scan,18F-FDG PET scan,and 11C-PiB PET scan,and the test results were analyzed.Results All three patients showed poor response to levodopa treatment and asymmetric dyskinesia including rigidity,tremor,dystonia and abnormalities in posture and gait.One patient showed apraxia and three patients presented with cognitive impairment.The crania MRI demonstrated mild cerebral atrophy which was slightly more severe in the contralateral side of the initially affected limb.The (18)F-FDG PET/CT scan revealed asymmetric decreased metabolism in the frontal,parietal,temporal,and insular lobe,as well as in basal ganglia.The results of (11)C-PiB PET showed that amyloid beta-peptide (A Beta) deposition in the cortex was positive in one patient,and negative in the other two patients.Conclusions Corticobasal syndrome is characterized by asymmetric dyskinesia and cognitive impairment,and often associated with apraxia,cortical sensory deficits,and alien limb phenomena.The MRI and FDG PET are helpful for CBS diagnosis,and the PiB PET facilitates the pathological diagnosis.%目的 探讨皮质基底节综合征的临床表现、影像学及病理学特点. 方法 3例患者均进行神经心理量表、头MRI、18F-FDG PET和11C-PIB PET检查.对3例皮质基底节综合征患者的临床、影像及病理资料进行总结分析. 结果 3例患者主要临床表现为不对称的运动障碍包括僵硬、震颤、肌张力障碍、姿势步态异常,对左旋多巴反应差,其中1例存在失用,3例患者均存在不同程度的认知功能障碍.头MRI显示轻度脑萎缩,以运动障碍起病肢体对侧稍明显.患者FDG PET显示不对称的额、顶、颞、岛叶及基底节代谢减低.PIB PET显示1例患者大脑皮层Aβ沉积阳性、2例阴性. 结论 皮质基底节综合征主要临床特征是不对称的运动障碍和认知功能障碍,常伴有失用、皮层感觉障碍和异己肢.头MRI和FDG PET有助于明确临床诊断,而PIB PET有有助于进一步明确病理诊断.