Luis C A, Barker W W, Gajaraj K, Harwood D, Petersen R, Kashuba A, Waters C, Jimison P, Pearl G, Petito C, Dickson D, Duara R
Wien Center for Alzheimer's Disease and Memory Disorders, Mount Sinai Medical Center, Miami Beach, FL 33141, USA.
Int J Geriatr Psychiatry. 1999 Jul;14(7):526-33.
To evaluate the sensitivity and specificity of the clinical features of three published diagnostic criteria for diffuse Lewy body disease (DLBD) using autopsy-confirmed Alzheimer's (AD), DLBD and AD+DLBD (mixed) dementia cases.
Retrospective chart review of an autopsy series of 56 patients selected from the State of Florida Brain Bank on the basis of a pathological diagnosis of either pure AD, DLBD (pure and common forms) or AD+DLBD (mixed) dementia. Clinical features were assessed by three raters blind to the pathological diagnosis.
The existing criteria for a clinical diagnosis of DLBD were highly specific (90-100%) but not very sensitive (49-63%) in the differential diagnosis of DLBD versus AD; sensitivity did improve (61-74%) when mixed AD+DLBD cases were eliminated. Clinical features that occur more frequently in DLBD than in AD were unspecified hallucinations, unspecified EPS, fluctuating course and rapid progression. Post-hoc analysis also indicated that hallucinations and EPS were more common early in the disease course of DLBD than in AD. Empirically derived criteria, formulated using the most prevalent clinical features, demonstrated sensitivity values of 57-96% for pure forms and 43-91% for mixed forms.
This study demonstrated that additional improvements in the established criteria for DLBD are needed. Our empirically derived criteria enhanced the distinction of DLBD from AD while allowing the clinician the choice of maximizing sensitivity with acceptable specificity, and vice versa.
使用经尸检确诊的阿尔茨海默病（AD）、路易体痴呆（DLBD）和AD合并DLBD（混合型）痴呆病例，评估三种已发表的弥漫性路易体病（DLBD）诊断标准临床特征的敏感性和特异性。
对从佛罗里达州脑库选取的56例尸检系列患者进行回顾性病历审查，这些患者基于纯AD、DLBD（纯型和常见型）或AD合并DLBD（混合型）痴呆的病理诊断。由三名对病理诊断不知情的评估者评估临床特征。
现有的DLBD临床诊断标准在DLBD与AD的鉴别诊断中具有高度特异性（90 - 100%），但敏感性不高（49 - 63%）；排除AD合并DLBD混合型病例后，敏感性有所提高（61 - 74%）。在DLBD中比在AD中更频繁出现的临床特征为未明确的幻觉、未明确的锥体外系症状（EPS）、病情波动和快速进展。事后分析还表明，幻觉和EPS在DLBD病程早期比在AD中更常见。根据最常见的临床特征制定的经验性标准显示，纯型的敏感性值为57 - 96%，混合型为43 - 91%。
本研究表明，DLBD既定标准需要进一步改进。我们的经验性标准增强了DLBD与AD的区分度，同时让临床医生能够选择在可接受的特异性下最大化敏感性，反之亦然。
