Update on frontotemporal dementia.
Update on frontotemporal dementia.
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DOI：
10.1097/nrl.0b013e3181b1d5c6
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发表时间：
2010-01
期刊：
影响因子：
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通讯作者：
Arvanitakis Z
中科院分区：
文献类型：
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作者：
Arvanitakis Z
额颞叶痴呆最近被认为是青年起病痴呆的常见原因。回顾目前额颞叶痴呆的临床评估、病理生理学的理解和治疗的方法。两种主要的临床表现是：1)行为，有冲动行为和去抑制，个性改变，如冷漠和冷漠，以及判断力差；2)语言，有不流利的失语失语伴失范(原发性进行性失语)，或流利失语伴早期词义丧失(语义性痴呆)。鉴别诊断包括其他神经退行性痴呆、血管和其他影响大脑的疾病以及精神疾病。包括神经心理测试以及结构和功能脑成像在内的研究可能有助于支持这一诊断。最近对病理生理学的了解进展表明，大多数病例存在潜在的泛素阳性包涵体，而一些病例存在tau阳性包涵体。基因突变，特别是在17号染色体上的tau或原颗粒基因，已经被识别出来。管理包括对症状性药物的试验和多方面的方法，包括改善环境和长期护理计划。对额颞叶痴呆症进行研究的医学研究人员的目标是找到治疗疾病的药物，并最终治愈这种毁灭性的疾病。
Frontotemporal dementia has recently been recognized as a common cause of young-onset dementia. To review the current approach to the clinical evaluation, understanding of pathophysiology, and management of frontotemporal dementia. Two main clinical presentations are: 1) behavioral, with impulsive behaviors and disinhibition, change in personality such as apathy and indifference, and poor judgment, and 2) language, with a non-fluent aphasia with anomia (primary progressive aphasia), or a fluent aphasia with early loss of word meaning (semantic dementia). The differential diagnosis includes other neurodegenerative dementias, vascular and other conditions affecting the brain, and psychiatric diseases. Investigations, including neuropsychological testing, and structural and functional brain imaging, may help support the diagnosis. Recent advances in understanding the pathophysiology have suggested that most cases have underlying ubiquitin-positive inclusions, while some have tau-positive inclusions. Genetic mutations, particularly on chromosome 17 in the tau or progranulin genes, have been identified. Management includes a trial of symptomatic medications and a multi-faceted approach, including environmental modification and long-term care planning. Medical researchers studying frontotemporal dementia aim to identify disease-modifying drugs and, ultimately, a cure for this devastating disease.