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阿尔茨海默病及相关痴呆症的临床神经病理学相关性

Clinical-neuropathological correlations in Alzheimer's disease and related dementias.

作者信息

Galasko D, Hansen L A, Katzman R, Wiederholt W, Masliah E, Terry R, Hill L R, Lessin P, Thal L J

机构信息

Alzheimer's Disease Research Center, University of California-San Diego.

出版信息

Arch Neurol. 1994 Sep;51(9):888-95. doi: 10.1001/archneur.1994.00540210060013.

DOI:10.1001/archneur.1994.00540210060013
PMID:8080388
Abstract

OBJECTIVE

To compare neurologists' initial clinical diagnoses made according to National Institute of Neurological and Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association (NINCDS-ADRDA) and Diagnostic and Statistical Manual of Mental Disorders, Revised Third Edition guidelines with neuropathological diagnoses of Alzheimer's disease (AD) and related dementias.

DESIGN

Consecutive autopsies in a prospective cohort study.

SETTING

Community-dwelling patients with dementia referred to neurologists at an Alzheimer's Disease Research Center and satellite clinics (n = 151) and patients initially evaluated when institutionalized (n = 19).

PATIENTS

Of 204 elderly patients who had an autopsy performed, 170 had received a complete dementia evaluation according to NINCDS-ADRDA guidelines.

MAIN OUTCOME MEASURES

Percentage agreement between neurologists' initial clinical diagnoses and pathological findings.

RESULTS

Of 137 patients clinically diagnosed as having probable or possible AD, 123 (90%) had AD neuropathological findings; this included 29 with AD accompanied by Lewy bodies, and 14 with AD and one or more infarcts. Cases of vascular and mixed dementia (AD and infarct[s]) had lower rates of agreement with pathological findings. Possible AD cases were more likely than probable AD cases to show pathological features other than AD. Clinicians predicted the presence or absence of AD pathological findings significantly better than chance. In patients with AD pathological lesions, older age of onset and male gender were significantly associated with shorter duration from disease onset to death.

CONCLUSIONS

Clinicians accurately predicted AD pathological findings or their absence in most cases. Attributing other degenerative dementias to AD, misdiagnosing patients with combined AD and Lewy bodies and misjudging the vascular contribution to dementia were the major areas of inaccuracy. Formal criteria for dementia associated with non-AD lesions, Lewy bodies, and infarcts need to be developed and tested.

摘要

目的

比较神经科医生依据美国国立神经疾病与中风研究所 - 阿尔茨海默病及相关疾病协会(NINCDS - ADRDA)和《精神疾病诊断与统计手册》第三版修订本指南做出的初始临床诊断与阿尔茨海默病(AD)及相关痴呆的神经病理学诊断。

设计

前瞻性队列研究中的连续尸检。

背景

转诊至阿尔茨海默病研究中心及卫星诊所的社区痴呆患者(n = 151)以及机构化时最初接受评估的患者(n = 19)。

患者

在204例接受尸检的老年患者中,170例根据NINCDS - ADRDA指南接受了全面的痴呆评估。

主要观察指标

神经科医生的初始临床诊断与病理结果之间的一致性百分比。

结果

在137例临床诊断为可能或疑似AD的患者中,123例(90%)有AD神经病理学表现;其中包括29例伴有路易体的AD患者,以及14例伴有AD和一处或多处梗死的患者。血管性痴呆和混合性痴呆(AD合并梗死)病例与病理结果的一致性率较低。疑似AD病例比很可能AD病例更有可能表现出AD以外的病理特征。临床医生对AD病理结果的存在与否的预测明显优于随机猜测。在有AD病理病变的患者中,发病年龄较大和男性与从发病到死亡的病程较短显著相关。

结论

临床医生在大多数情况下准确预测了AD病理结果的存在与否。将其他退行性痴呆归因于AD、误诊合并AD和路易体的患者以及错误判断血管对痴呆的影响是不准确的主要方面。需要制定并测试与非AD病变、路易体和梗死相关的痴呆的正式标准。

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