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额颞叶变性:临床诊断标准的共识

Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria.

作者信息

Neary D, Snowden J S, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert P H, Albert M, Boone K, Miller B L, Cummings J, Benson D F

机构信息

Manchester Royal Infirmary, UK.

出版信息

Neurology. 1998 Dec;51(6):1546-54. doi: 10.1212/wnl.51.6.1546.

DOI:10.1212/wnl.51.6.1546
PMID:9855500
Abstract

OBJECTIVE

To improve clinical recognition and provide research diagnostic criteria for three clinical syndromes associated with frontotemporal lobar degeneration.

METHODS

Consensus criteria for the three prototypic syndromes-frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia-were developed by members of an international workshop on frontotemporal lobar degeneration. These criteria build on earlier published clinical diagnostic guidelines for frontotemporal dementia produced by some of the workshop members.

RESULTS

The consensus criteria specify core and supportive features for each of the three prototypic clinical syndromes and provide broad inclusion and exclusion criteria for the generic entity of frontotemporal lobar degeneration. The criteria are presented in lists, and operational definitions for features are provided in the text.

CONCLUSIONS

The criteria ought to provide the foundation for research work into the neuropsychology, neuropathology, genetics, molecular biology, and epidemiology of these important clinical disorders that account for a substantial proportion of cases of primary degenerative dementia occurring before the age of 65 years.

摘要

目的

提高对与额颞叶变性相关的三种临床综合征的临床认识,并提供研究诊断标准。

方法

额颞叶变性国际研讨会的成员制定了三种典型综合征——额颞叶痴呆、进行性非流利性失语和语义性痴呆——的共识标准。这些标准基于该研讨会部分成员早期发表的额颞叶痴呆临床诊断指南。

结果

共识标准明确了三种典型临床综合征各自的核心特征和支持性特征,并为额颞叶变性这一通用实体提供了广泛的纳入和排除标准。标准以列表形式呈现,文中还提供了各特征的操作定义。

结论

这些标准应为对这些重要临床疾病的神经心理学、神经病理学、遗传学、分子生物学和流行病学开展研究工作奠定基础,这些疾病在65岁之前发生的原发性退行性痴呆病例中占相当大的比例。

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