皮质基底节变性综合征四例临床及影像学分析

Clinical and neuroimaging analysis of 4 patients with corticobasal degeneration syndrome

目的探讨皮质基底节变性综合征的临床特征、影像学表现和治疗。方法对4例临床诊断皮质基底节变性患者的资料进行回顾性分析。结果4例患者的首发症状均为单侧肢体的帕金森样表现，对左旋多巴治疗无反应，4例均有肢体失用、构音障碍，3例伴肌阵挛，2例伴有痴呆，1例伴不自主运动。4例患者头部MRI扫描均显示受累肢体对侧脑皮质萎缩，以额顶叶显著；头葡萄糖代谢正电子发射断层扫描（FDG—PET）检查均显示不对称的大脑皮质和基底节区葡萄糖代谢减低。结论皮质基底节变性综合征患者的临床特征为不对称的帕金森样表现，可伴有认知功能障碍、失用、构音障碍、肌阵挛和不自主运动等症状；头MRI、FDG—PET检查有助于诊断；目前尚无有效治疗方法。

Objective To investigate clinical and imaging features of corticobasal degeneration syndrome （ CBDS ）. Methods A clinical, imaging and therapeutic analysis of 4 cases of clinically diagnosed with corticobasal degeneration was conducted. Results Asymmetric parkinsonism was the first symptom in all 4 cases who lacked of response to levodopa. Other symptoms including limb apraxia and anarthria occurred in all 4 cases, myoclonus occurred in three, dementia occurred in two, and involuntary movement occurred in one. All the patients had asymmetric frontoparietal cortical atrophy in the eontra- lateral to the dominantly affected limbs on MRI. Asymmetric hypometabolism of the frontoparietal cortex and basal ganglia was observed on 18F-FDG PET in all 4 cases. Conclusions The clinical features of CBDS are asymmetric parkinsonism, dementia, apraxia, anarthria, myoclonus, and involuntary movement. Brain MRI and 18F-FDG PET are helpful to diagnosis of CBDS. There is no effective treatment for CBDS at this time.