Mega M S, Masterman D L, Benson D F, Vinters H V, Tomiyasu U, Craig A H, Foti D J, Kaufer D, Scharre D W, Fairbanks L, Cummings J L
Department of Neurology, UCLA School of Medicine 90025, USA.
Neurology. 1996 Dec;47(6):1403-9. doi: 10.1212/wnl.47.6.1403.
Clinical criteria for dementia with Lewy bodies (DLB) have been proposed, but their formulation, reliability, and validity require further study. Pathologic criteria for DLB are also undergoing evolution. Two studies were conducted with the goal of identifying the components of these evolving criteria that may benefit from further refinement; one study evaluated the components of the clinical criteria and another study operationalized the pathologic criteria for DLB. Twenty-four patients with a premorbid diagnosis of probable or possible Alzheimer's disease (AD) (n = 18), Parkinson's disease (PD) (n = 5), or progressive supranuclear palsy (PSP) (n = 1) were studied. Inter-rater reliability and validity of the clinical criteria were determined by a retrospective chart review, done by five neurologists, and a blinded pathologic evaluation. The Consortium on dementia with Lewy bodies (CDLB) pathologic criteria were operationalized to compare past criteria and test the validity of the evolving clinical criteria on the dementia patients. Three or more cortical fields (at 250 x magnification) with many (four or more) Lewy bodies (LBs) on ubiquitin immunoreactive sections were required to meet the CDLB neocortical score of > 6. Fifteen of the AD patients had at least one LB in a cortical section, four had many LBs, while three had no LBs; all patients with movement disorder had at least one LB in a cortical section. The sensitivity/specificity ratio of the CDLB probable DLB clinical criteria based upon many LBs being present was 75%/79%. Reformulated clinical criteria that require the presence of extrapyramidal signs significantly predicted those patients with many LBs versus those with few or no LBs (chi 2 = 5.48, p = 0.02) and increased clinical specificity to 100%. This preliminary study identifies components of the evolving clinical and pathologic criteria for DLB that require further refinement.
路易体痴呆（DLB）的临床标准已被提出，但其制定、可靠性和有效性仍需进一步研究。DLB的病理标准也在不断演变。进行了两项研究，目的是确定这些不断演变的标准中哪些成分可能需要进一步完善；一项研究评估了临床标准的成分，另一项研究则对DLB的病理标准进行了操作化定义。对24例病前诊断为可能或疑似阿尔茨海默病（AD）（n = 18）、帕金森病（PD）（n = 5）或进行性核上性麻痹（PSP）（n = 1）的患者进行了研究。通过五位神经科医生进行的回顾性病历审查和盲法病理评估，确定了临床标准的评分者间信度和效度。对路易体痴呆联盟（CDLB）的病理标准进行了操作化定义，以比较过去的标准，并检验不断演变的临床标准对痴呆患者的有效性。在泛素免疫反应切片上，三个或更多皮质区域（放大250倍）有许多（四个或更多）路易小体（LB），才能达到CDLB新皮质评分> 6。15例AD患者的皮质切片中至少有一个LB，4例有许多LB，而3例没有LB；所有运动障碍患者的皮质切片中至少有一个LB。基于存在许多LB的CDLB可能DLB临床标准的敏感性/特异性比为75%/79%。重新制定的临床标准要求存在锥体外系体征，这显著预测了有许多LB的患者与有少量或没有LB的患者（χ2 = 5.48，p = 0.02），并将临床特异性提高到100%。这项初步研究确定了DLB不断演变的临床和病理标准中需要进一步完善的成分。
