| 摘要: |
| 额颞叶变性（frontotemporal lobar degeneration，FTLD）是以局限性额叶／颞叶变性为特征的非阿尔茨海默病（Alzheimer Fdisease，AD）痴呆综合征。近年的研究发现它与tau蛋白的变性有关，并与皮质基底节变性（corticobasal ganglionic degeneration，CBD）、进行性核上性麻痹（progressive supranuclear palsy，PSP）、运动神经元病（motor neuron disease，MND）相关或重叠。本文就FTLD的病因、病理、遗传学、临床分型以及诊断标准等方面的研究进展作一综述 |
| 关键词: 额颞叶变性 tau蛋白 病因 病理学 诊断 |
| DOI：10.3724/SP.J.1008.2007.00204 |
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| 基金项目: |
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| Fronotemporal lobar degeneration： an update |
| WU Jian-xin ,CHEN Xian-wen |
| (1. Department of Neurology, No. 117 Hospital of PLA, Hangzhou 310013, China; 2. Department of Neurology, the First Affiliated Hospital of Anhui Medical University, Hefei 230022) |
| Abstract: |
| Frontotemporal lobar degeneration （FTLD） is a non-Alzheimer dementia syndrome characterized by focal atrophy of the frontal and/or temporal lobes. Recently, it has been found that FTLD is related to the degeneration of tau protein, and is closely associated with corticobasal ganglionic degeneration, progressive supranuclear palsy, and motor neuron disease. This article reviews the progress in etiology, genetics, pathology, clinical features, and diagnostic criteria of FTLD |
| Key words: frontotemporal lobar degeneration tau protein etiology pathology diagnosis |
