Varma A R, Snowden J S, Lloyd J J, Talbot P R, Mann D M, Neary D
Department of Neurology, Manchester Royal Infirmary, UK.
J Neurol Neurosurg Psychiatry. 1999 Feb;66(2):184-8. doi: 10.1136/jnnp.66.2.184.
The diagnosis of Alzheimer's disease (AD) is now reliant on the use of NINCDS-ADRDA criteria. Other diseases causing dementia are being increasingly recognised--for example, frontotemporal dementia (FTD). Historically, these disorders have not been clearly demarcated from AD. This study assesses the capability of the NINCDS-ADRDA criteria to accurately distinguish AD from FTD in a series of pathologically proved cases.
The case records of 56 patients (30 with AD, 26 with FTD) who had undergone neuropsychological evaluation, brain imaging, and ultimately postmortem, were assessed in terms of whether at initial diagnosis the NINCDS-ADRDA criteria were successful in diagnosing those patients who had AD and excluding those who did not.
(1) The overall sensitivity of the NINCDS-ADRDA criteria in diagnosing "probable" AD from 56 patients with cortical dementia (AD and FTD) was 0.93. However, the specificity was only 0.23; most patients with FTD also fulfilled NINCDS-ADRDA criteria for AD. (2) Cognitive deficits in the realms of orientation and praxis significantly increased the odds of a patient having AD compared with FTD, whereas deficits in problem solving significantly decreased the odds. Neuropsychological impairments in the domains of attention, language, perception, and memory as defined in the NINCDS-ADRDA statement did not contribute to the clinical differentiation of AD and FTD.
NINCDS-ADRDA criteria fail accurately to differentiate AD from FTD. Suggestions to improve the diagnostic specificity of the current criteria are made.
目前阿尔茨海默病（AD）的诊断依赖于使用美国国立神经疾病和中风研究所 - 阿尔茨海默病及相关疾病协会（NINCDS - ADRDA）标准。越来越多其他导致痴呆的疾病被认识到，例如额颞叶痴呆（FTD）。从历史上看，这些疾病与AD并未明确区分。本研究评估了NINCDS - ADRDA标准在一系列经病理证实的病例中准确区分AD与FTD的能力。
对56例患者（30例AD患者，26例FTD患者）的病例记录进行评估，这些患者均接受了神经心理学评估、脑成像检查并最终进行了尸检，评估内容为在初始诊断时NINCDS - ADRDA标准是否成功诊断出患有AD的患者并排除未患AD的患者。
（1）NINCDS - ADRDA标准从56例皮质性痴呆（AD和FTD）患者中诊断“可能”AD的总体敏感性为0.93。然而，特异性仅为0.23；大多数FTD患者也符合AD的NINCDS - ADRDA标准。（2）与FTD相比，定向和运用领域的认知缺陷显著增加了患者患AD的几率，而解决问题方面的缺陷则显著降低了这种几率。NINCDS - ADRDA声明中定义的注意力、语言、感知和记忆领域的神经心理学损害对AD和FTD的临床鉴别没有帮助。
NINCDS - ADRDA标准未能准确区分AD与FTD。本文提出了提高当前标准诊断特异性的建议。
