Chow Tiffany W, Alobaidy Ammar A
Baycrest Rotman Research Institute, 3560 Bathurst Street, 8th Floor Brain Health Complex, Toronto, ON M6A 2E1, Canada.
Continuum (Minneap Minn). 2013 Apr;19(2 Dementia):438-56. doi: 10.1212/01.CON.0000429173.35439.9c.
Within the continuously growing body of knowledge in the field of dementia, frontotemporal degeneration stands out in importance as the second most common cause of early-onset dementia after Alzheimer disease. Neurologists, neuropsychologists, and speech pathologists are particularly involved in the diagnosis and recognition of etiologies for patients with deficits in frontal lobe function and language.
The recent discovery of a novel mutant gene (C9ORF72) and the new nomenclature adopted for subclassification have significantly promoted our understanding of this disorder.
This article relates the most recent consensus criteria for diagnosis of the two forms of frontotemporal degeneration (ie, behavioral and primary progressive aphasia variants) to basic neurologic principles and remind clinicians of the neuropsychiatric and neuroradiologic components that clarify frontotemporal degeneration diagnoses and guide management.
在痴呆领域不断增长的知识体系中，额颞叶变性作为仅次于阿尔茨海默病的第二大常见早发性痴呆病因，具有重要意义。神经科医生、神经心理学家和言语病理学家尤其参与到对额叶功能和语言功能有缺陷患者病因的诊断和识别中。
一种新型突变基因（C9ORF72）的最近发现以及用于亚分类的新命名法显著促进了我们对这种疾病的理解。
本文将额颞叶变性两种形式（即行为变异型和原发性进行性失语变异型）诊断的最新共识标准与基本神经学原理相关联，并提醒临床医生注意那些有助于明确额颞叶变性诊断并指导治疗的神经精神和神经放射学组成部分。
