Frontotemporal dementias: a review.

Frontotemporal dementias: a review.
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DOI:
10.1186/1744-859x-6-15
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发表时间:
2007-06-12
影响因子:
3.7
通讯作者:
Tampi, Rajesh R
Tampi, Rajesh R
中科院分区:
医学3区
文献类型:
--
作者:
Weder, Natalie D;Aziz, Rehan;Tampi, Rajesh R

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痴呆是一种临床状态,其特征在于多个认知领域的功能丧失。就个人痛苦和经济损失而言,这是一种代价高昂的疾病。额颞叶痴呆(FTD)是目前首选的术语,而不是皮克病,用于描述以大脑额叶和前颞区局灶性萎缩为特征的非阿尔茨海默氏痴呆。FTD的患病率相当高,尽管不同研究中的具体数字有所不同。它通常发生在35-75岁的年龄范围内,在有痴呆症家族史的人中更常见。与该疾病相关的风险因素包括头部损伤和FTD家族史。尽管关于不同类型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)额叶或行为变体(FvFTD),2)颞叶失语变体,也称为语义性痴呆(SD),和3)进行性失语(PA)。这些不同的变体在临床表现、认知缺陷和受影响的大脑区域方面有所不同。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,以确认和阐明诊断。治疗这种实体包括行为和药理学方法。药物如5-羟色胺再摄取抑制剂、抗精神病药、情绪稳定剂和其他新型治疗已用于FTD,成功率不同。进一步的研究应致力于了解和开发新的诊断和治疗模式,以改善患者的预后和生活质量。
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.