额颞叶痴呆
Frontotemporal Dementias.
作者信息
Finger Elizabeth C
出版信息
Continuum (Minneap Minn). 2016 Apr;22(2 Dementia):464-89. doi: 10.1212/CON.0000000000000300.
PURPOSE OF REVIEW
This article reviews the common behavioral and cognitive features of frontotemporal dementia (FTD) and related disorders as well as the distinguishing clinical, genetic, and pathologic features of the most common subtypes.
RECENT FINDINGS
Advances in clinical phenotyping, genetics, and biomarkers have enabled improved predictions of the specific underlying molecular pathology associated with different presentations of FTD. Evaluation of large international cohorts has led to recent refinements in diagnostic criteria for several of the FTD subtypes.
SUMMARY
The FTDs are a group of neurodegenerative disorders featuring progressive deterioration of behavior or language and associated pathology in the frontal or temporal lobes. Based on anatomic, genetic, and neuropathologic categorizations, the six clinical subtypes of FTD or related disorders are: (1) behavioral variant of FTD, (2) semantic variant primary progressive aphasia, (3) nonfluent agrammatic variant primary progressive aphasia, (4) corticobasal syndrome, (5) progressive supranuclear palsy, and (6) FTD associated with motor neuron disease. Recognition and accurate diagnoses of FTD subtypes will aid the neurologist in the management of patients with FTD.
综述目的
本文综述额颞叶痴呆（FTD）及相关疾病的常见行为和认知特征，以及最常见亚型的鉴别性临床、遗传和病理特征。
最新发现
临床表型分析、遗传学和生物标志物方面的进展使得对与FTD不同表现相关的特定潜在分子病理学的预测得到改善。对大型国际队列的评估导致近期对几种FTD亚型的诊断标准进行了完善。
总结
FTD是一组神经退行性疾病，其特征为行为或语言进行性恶化以及额叶或颞叶相关病理学改变。基于解剖学、遗传学和神经病理学分类，FTD或相关疾病的六种临床亚型为：（1）FTD行为变异型，（2）语义变异型原发性进行性失语，（3）非流利性语法缺失变异型原发性进行性失语，（4）皮质基底节综合征，（5）进行性核上性麻痹，以及（6）与运动神经元病相关的FTD。FTD亚型的识别和准确诊断将有助于神经科医生对FTD患者进行管理。
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