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额颞叶痴呆:综述

Frontotemporal dementias: a review.

作者信息

Weder Natalie D, Aziz Rehan, Wilkins Kirsten, Tampi Rajesh R

机构信息

Department of Psychiatry, Yale University School of Medicine, New Haven, CT, USA.

出版信息

Ann Gen Psychiatry. 2007 Jun 12;6:15. doi: 10.1186/1744-859X-6-15.

Abstract

Dementia is a clinical state characterized by loss of function in multiple cognitive domains. It is a costly disease in terms of both personal suffering and economic loss. Frontotemporal dementia (FTD) is the term now preferred over Picks disease to describe the spectrum of non-Alzheimers dementias characterized by focal atrophy of the frontal and anterior temporal regions of the brain. The prevalence of FTD is considerable, though specific figures vary among different studies. It occurs usually in an age range of 35-75 and it is more common in individuals with a positive family history of dementia. The risk factors associated with this disorder include head injury and family history of FTD. Although there is some controversy regarding the further syndromatic subdivision of the different types of FTD, the three major clinical presentations of FTD include: 1) a frontal or behavioral variant (FvFTD), 2) a temporal, aphasic variant, also called Semantic dementia (SD), and 3) a progressive aphasia (PA). These different variants differ in their clinical presentation, cognitive deficits, and affected brain regions. Patients with FTD should have a neuropsychiatric assessment, neuropsychological testing and neuroimaging studies to confirm and clarify the diagnosis. Treatment for this entity consists of behavioral and pharmacological approaches. Medications such as serotonin reuptake inhibitors, antipsychotics, mood stabilizer and other novel treatments have been used in FTD with different rates of success. Further research should be directed at understanding and developing new diagnostic and therapeutic modalities to improve the patients' prognosis and quality of life.

摘要

痴呆是一种以多个认知领域功能丧失为特征的临床状态。就个人痛苦和经济损失而言,它是一种代价高昂的疾病。额颞叶痴呆(FTD)是目前比皮克病更常用的术语,用于描述以大脑额叶和颞叶前部局灶性萎缩为特征的非阿尔茨海默病性痴呆谱系。FTD的患病率相当可观,尽管不同研究中的具体数字有所不同。它通常发生在35 - 75岁的年龄段,在有痴呆家族史阳性的个体中更为常见。与这种疾病相关的危险因素包括头部损伤和FTD家族史。尽管对于不同类型FTD的进一步综合征细分存在一些争议,但FTD的三种主要临床表现包括:1)额叶或行为变异型(FvFTD),2)颞叶失语变异型,也称为语义性痴呆(SD),以及3)进行性失语(PA)。这些不同的变异型在临床表现、认知缺陷和受影响的脑区方面存在差异。FTD患者应进行神经精神评估、神经心理学测试和神经影像学研究,以确认和明确诊断。针对这种疾病的治疗包括行为和药物治疗方法。诸如5-羟色胺再摄取抑制剂、抗精神病药物、心境稳定剂和其他新型治疗方法已用于FTD,成功率各不相同。进一步的研究应致力于理解和开发新的诊断和治疗方法,以改善患者的预后和生活质量。

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