Tiraboschi Pietro, Salmon David P, Hansen Lawrence A, Hofstetter Richard C, Thal Leon J, Corey-Bloom Jody
Dipartimento di Scienze Neurologiche, Ospedale Niguarda Ca' Granda, Milano, Italy.
Brain. 2006 Mar;129(Pt 3):729-35. doi: 10.1093/brain/awh725. Epub 2006 Jan 9.
To determine which clinical feature(s) [among visual hallucinations (VH), extrapyramidal signs (EPS) and visuospatial impairment] in the earliest stages of disease best predicted a diagnosis of dementia with Lewy bodies (DLB) at autopsy, first-visit data of 23 pathologically proven DLB and 94 Alzheimer's disease cases were compared. There were no group differences with regard to age, gender, education or global severity of dementia at presentation (mean Mini-Mental State Examination: 24.0 versus 25.0, mean Dementia Rating Scale: 123.6 versus 125.7). DLB patients at initial presentation displayed an increased frequency of VH (P = 0.001), but not EPS (P = 0.3), compared to Alzheimer's disease patients. However, only a minority of DLB cases had either VH (22%), EPS (26%) or both (13%). In contrast, although not a core feature, visuospatial/constructional impairment was observed in most of the DLB cases (74%). Among clinical variables, presence/recent history of VH was the most specific to DLB (99%), and visuospatial impairment was the most sensitive (74%). As a result, VH at presentation were the best positive predictor of DLB at autopsy (positive predictive value: 83% versus 32% or less for all other variables), while lack of visuospatial impairment was the best negative predictor (negative predictive value: 90%). We conclude that the best model for differentiating DLB from Alzheimer's disease in the earliest stages of disease includes VH and visuospatial/constructional dysfunction, but not spontaneous EPS, as predictors. This suggests that clinical history plus a brief assessment of visuospatial function may be of the greatest value in correctly identifying DLB early during the course of disease.
为了确定疾病早期阶段的哪些临床特征(视幻觉、锥体外系症状和视觉空间障碍)最能预测尸检时路易体痴呆(DLB)的诊断,比较了23例经病理证实的DLB患者和94例阿尔茨海默病患者的首次就诊数据。在年龄、性别、教育程度或就诊时痴呆的总体严重程度方面,两组没有差异(简易精神状态检查表平均分:24.0对25.0;痴呆评定量表平均分:123.6对125.7)。与阿尔茨海默病患者相比,DLB患者初诊时视幻觉出现频率增加(P = 0.001),但锥体外系症状未增加(P = 0.3)。然而,只有少数DLB病例有视幻觉(22%)、锥体外系症状(26%)或两者都有(13%)。相比之下,虽然不是核心特征,但大多数DLB病例(74%)存在视觉空间/结构障碍。在临床变量中,视幻觉的存在/近期病史对DLB最具特异性(99%),视觉空间障碍最敏感(74%)。因此,初诊时的视幻觉是尸检时DLB的最佳阳性预测指标(阳性预测值:83%,而所有其他变量为32%或更低),而无视觉空间障碍是最佳阴性预测指标(阴性预测值:90%)。我们得出结论,在疾病早期阶段将DLB与阿尔茨海默病区分开来的最佳模型包括视幻觉和视觉空间/结构功能障碍,但不包括自发性锥体外系症状作为预测指标。这表明临床病史加上对视空间功能的简短评估在疾病过程早期正确识别DLB方面可能最有价值。