路易体痴呆八例临床分析

Clinical analysis of 8 dementia with Lewy bodies

目的探讨路易体痴呆的临床特征,诊断和治疗.方法对8例临床诊断的路易体痴呆患者的资料进行回顾性分析.结果 8例患者的首发症状均为波动性认知功能障碍,伴随持续存在的视幻觉和帕金森综合征表现.主要为中度痴呆,视空结构能力损害明显;波动性认知功能障碍(FC)评分为4～10,平均7.4±2.1,为轻至中度波动;统一帕金森病等级量表(UPDRS)评分为31～71,平均47.8±14.3.1例有快眼动相(REM)睡眠行为障碍的患者REM睡眠期颏下肌电图出现大量电活动.8例患者头部MRI及CT扫描均显示脑萎缩.1例脑单光子发射计算机体层摄影术(SPECT)检查显示纹状体多巴胺功能受损.左旋多巴和胆碱酯酶抑制剂盐酸多奈哌齐治疗有效.结论路易体痴呆的临床特征为波动性认知功能障碍、持续存在的视幻觉和锥体外系运动功能障碍,诊断以病史、神经心理测试和影像学检查为依据,左旋多巴、胆碱酯酶抑制剂可分别改善患者的锥体外系症状和认知功能障碍.

Objective To investigate the clinical features,diagnosis and treatment of dementia with Lewy bodies(DLB).Methods A clinical, neuropsychological ,neuroimaging and therapeutic analysis on 8 cases of clinically diagnosed dementia with Lewy bodies was conducted.Results Fluctuating cognition(FC) was the first symptom in all 8 patients,followed by persistent visual hallucinations and Parkinsonism. Patients showed moderate dementia and severe constructional disabilities .The ratings of FC were mild to moderate with scores 4-10(mean 7.4±2.1). The UPDRS scores ranged from 31 to 71 (mean 47.8±14.3). The polysomnography of the patient with REM sleep behavior disorder showed augmented submental muscle activity during REM and no EEG epileptiform activity. MRI or CT scans showed global brain atrophy in all 8 patients. SPECT of Sleep patient showed impaired striatal dopaminergic function . L-dopa and donepezil therapy were effective.Conclusions The main clinical features of DLB are fluctuating cognition, persistent visual hallucinations and extrapyramidal motor symptoms. The diagnosis relies on history and current symptoms, neuropsychological testing and findings of neuroimaging. L-dopa and cholinesterase-inhibitor drugs can improve mobility and cognitive symptoms in DLB.