Frontotemporal dementia.

Frontotemporal dementia.
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DOI:
10.1016/s0140-6736(15)00461-4
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发表时间:
2015-10-24
期刊:
Lancet (London, England)
影响因子:
--
通讯作者:
Miller BL
Miller BL
中科院分区:
其他
文献类型:
--
作者:
Bang J;Spina S;Miller BL

文献摘要

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额颞叶痴呆是一个总括性临床术语,包括一组神经退行性疾病,其特征是行为、执行功能或语言的进行性缺陷。额颞叶痴呆是一种常见的痴呆类型,特别是在65岁以下的患者中。这种疾病可以模仿许多精神疾病,因为突出的行为特征。各种潜在的神经病理实体导致额颞叶痴呆的临床表型,所有这些的特征在于额叶和颞叶皮质的选择性变性。遗传是额颞叶痴呆的一个重要危险因素。临床、影像学和分子表征的进展提高了额颞叶痴呆诊断的准确性,从而使这些综合征与精神疾病的准确区分成为可能。随着对额颞叶痴呆的分子基础的理解的提高,合理的治疗方法开始出现。
Frontotemporal dementia is an umbrella clinical term that encompasses a group of neurodegenerative diseases characterised by progressive deficits in behaviour, executive function, or language. Frontotemporal dementia is a common type of dementia, particularly in patients younger than 65 years. The disease can mimic many psychiatric disorders because of the prominent behavioural features. Various underlying neuropathological entities lead to the frontotemporal dementia clinical phenotype, all of which are characterised by the selective degeneration of the frontal and temporal cortices. Genetics is an important risk factor for frontotemporal dementia. Advances in clinical, imaging, and molecular characterisation have increased the accuracy of frontotemporal dementia diagnosis, thus allowing for the accurate differentiation of these syndromes from psychiatric disorders. As the understanding of the molecular basis for frontotemporal dementia improves, rational therapies are beginning to emerge.