Frontotemporal dementia
Frontotemporal dementia
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DOI：
10.1097/01.wco.0000247606.57567.41
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发表时间：
2006-12-01
影响因子：
4.8
通讯作者：
Hodges, John R.
中科院分区：
文献类型：
--
作者：
Knibb, Jonathan A.;Kipps, Christopher M.;Hodges, John R.
综述的目的额颞叶变性综合征越来越被认为是早发性痴呆的重要原因。诊断共识标准已经建立了近十年，并形成了其临床分类的框架。虽然这些标准仍然是有用的，越来越多的证据表明，修订可能是必要的，以提高其有效性和适用性。最近的研究结果在每一个单独的综合征，核心特征是不一致的，目前用于排除一个条件，如情节记忆受损的标准，往往存在。然而，影像学检查可能会增加诊断的重要性，特别是对语义性痴呆的诊断和行为综合征的预后。这些综合征之间存在临床和病理重叠，但进行性非流利性失语和语义性痴呆之间的临床区别正在加强。几个系列已经完善了我们对临床综合征和组织病理学亚型之间的对应关系的理解：对于taunegative，泛素阳性形式很强，而对于tau阳性形式更易变，但前瞻性研究仍然很少。遗传因素的影响在不同的综合征之间有很大的差异。总结进一步的研究应该旨在整合详细的临床，放射学，病理学和遗传学信息。
Purpose of review The syndromes of frontotemporal lobar degeneration are increasingly recognized as an important cause of early-onset dementia. Diagnostic consensus criteria have now been established for almost a decade, and form the framework for its clinical classification. While these criteria remain useful, a growing body of evidence suggests that revisions may be necessary to improve their validity and applicability.Recent findings In each individual syndrome, the core features are not uniformly present, and criteria that are currently used to exclude a condition, such as impaired episodic memory, are often present. Imaging, however, may warrant increased diagnostic prominence, particularly for diagnosis in semantic dementia and prognosis in behavioural syndromes. There is clinical and pathological overlap between the syndromes, but the clinical distinction between progressive nonfluent aphasia and semantic dementia is strengthening. Several series have refined our understanding of the correspondence between clinical syndromes and histopathological subtype: strong for taunegative, ubiquitin-positive forms and more variable for taupositive forms, yet prospective studies are still rare. The influence of genetic factors varies substantially across the syndromes.Summary Further research should aim to integrate detailed clinical, radiological, pathological and genetic information.