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摘要
背景:额颞叶痴呆(FTD)是一组异质性疾病,是早发性痴呆的第二大原因,使其成为继承性病例数最多的病因 综述摘要:FTD的特点是临床,遗传和组织病理学特征具有相当大的差异性。 患者可能出现症状,从行为障碍到不同的语言障碍,伴有或不伴有运动神经元紊乱或相关的帕金森综合征。 额叶和颞叶萎缩是最相关的放射学发现。 在过去的10年中,这一临床实体的知识在遗传学和组织病理学上都经历了显着的变化,这些变化有助于建立更一致的临床标准。 到目前为止,已经描述了导致FTLD的10个基因,并且已经在聚集体中检测到导致萎缩的四种不同蛋白质。 结论:本综述主要针对临床医师,旨在提供这些神经退行性疾病的基本知识,并阐明复杂的FTD情况。
关键词: FTLD,表型,蛋白质,基因型,复习,神经科医生。
Current Alzheimer Research
Title:Frontotemporal Lobar Degeneration (FTLD): Review and Update for Clinical Neurologists
Volume: 15 Issue: 6
关键词: FTLD,表型,蛋白质,基因型,复习,神经科医生。
摘要: Background: Frontotemporal Dementia (FTD) is a heterogeneous group of disorders and the second most frequent cause of early onset dementia making it the highest number of inherited cases.
Review Summary: FTD is characterized by considerable variability in clinical, genetic and histopathologic features. Patients may present symptoms ranging from behavioural disturbances to different language disorders, with or without motor neuron disorders or associated parkinsonism. Atrophy in frontal and temporal lobes is the most relevant radiological finding. In the last 10 years, the knowledge of this clinical entity has undergone remarkable changes both genetically and histopathologically, which have served to establish more consistent clinical criteria. Until now, 10 genes causative of FTLD have been described and up to four different proteins causative of atrophy have been detected in aggregates.
Conclusion: This review is mostly addressed to clinicians and aims to provide basic knowledge of these neurodegenerative disorders and clarify the complex FTD scenario.
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Cite this article as:
Frontotemporal Lobar Degeneration (FTLD): Review and Update for Clinical Neurologists, Current Alzheimer Research 2018; 15 (6) . https://dx.doi.org/10.2174/1567205014666170725130819
DOI https://dx.doi.org/10.2174/1567205014666170725130819 |
Print ISSN 1567-2050 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5828 |
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