43例路易体痴呆患者的临床及影像学特点分析

Clinical and neuroimaging features of 43 cases of dementia with Lewy bodies

目的探讨路易体痴呆患者的临床特征及影像学特点。方法对2011年6月至2015年6月在天津市环湖医院诊断的43例路易体痴呆患者的临床和影像学资料进行回顾性分析。结果43例患者中男24例（55．8％），女19例（44．2％），年龄50～89岁，平均发病年龄（69．9±9．0）岁。首发症状中包含认知功能障碍者占67．4％，幻觉者占25．6％，帕金森症状者占18．600。临床表现中所有患者均表现出认知功能障碍，88．4％患者的认知障碍呈波动性。86．0%的患者伴随帕金森症状，76．7％患者出现幻觉。在支持症状中，74．4％患者有精神症状，34．9％有自主神经功能障碍，32．6％有短暂意识丧失，34．9％有反复跌倒，14．0％的患者对抗精神病药或神经镇静药高度敏感。43例患者头MRI和/或CT均显示弥漫性脑皮层萎缩，34．9％的患者海马萎缩，且海马萎缩与皮层萎缩程度相一致。12例患者行头部^18F—FDG PET-CT检查，均表现为枕叶、顶枕或颞顶枕联合区代谢减低。PiB—PET—CT检查均显示双侧额颞顶枕皮层淀粉样蛋白沉积。随访中13例患者已卧床，从起病到卧床平均（6．0±2．6）年。结论路易体痴呆发病年龄较晚，以认知功能障碍起病者最为常见。波动性、幻觉、帕金森样症状、精神症状等较为突出。多进展迅速，卧床出现早。结构MRI没有特异性，PET-CT可能能为鉴别诊断提供线索。

Objective To explore the clinical and neuroimaging features of dementia with Lewy bodies（DLB）. Methods Clinical and neuroimaging data of 43 patients diagnosed as DLB in Tianjin Huanhu hospital from June 2011 to June 2015 were retrospectively analyzed. Results The 43 DLB patients with 24 males（55.8%）and 19 females（44.2%）aged 50 89 years,mean age of onset （69.9±9.0）years served as subjects. In the onset [eatures of DLB, 64.4% cases represented as cognitive impairment, 25.6% as hallucinations, and 18.6% as Parkinsonism. In the clinical manifestation, all patients showed cognitive impairment, 88.4% of patients showed fluctuating cognition, 86.0% showed Parkinsonism and 76.7% showed visual hallucinations. As for the supportive symptoms of DLB, 74.4% of patients displayed psychiatric symptom, 34.9% displayed autonomic dysfunction, 32.6% displayed transient loss of consciousness, 34.9% displayed repeated falls, and 14.0% displayed severe sensitivity to antipsychotic or sedative drugs. The data from MRI/CT scan suggested that all patients displayed diffuse cerebral atrophy, with 34.9 % of the patients showing hippocampus atrophy. The atrophy of hippocampus was consistent with the cerebral atrophy. 12 patients underwent ^18F- FDG PET scan and ^11C-PiB PET scan. The results from ^18F-FDG PET showed hypo metabolism in the occipital lobe, parieto occipital region or temporo-parieto occipital region. Moreover, the results from ^11 C-PiB PET indicated Aβ deposition in bilateral frontal, temporal, parietal and occipital cortex. During the follow up, 13 patients had to stay in bed, and the average time from the onset to being bedridden was（6.0 ± 2.6）years. Conclusions DLB is a late onset disease, in which the cognitive impairment is most common. Clinical symptoms of DLB are characterized by cognitive fluctuation, hallucinations, Parkinsonism, psychiatric symptom, et al. DLB usually progresses rapidly, and the patients have to stay in bed on the early stage of the disease. Structural MRI imaging is non-specific, however, PET scan might be helpful for the diagnosis of DLB .