Arvanitakis Zoe
Department of Neurological Sciences, Rush Alzheimer Disease Center, Chicago, IL 60612, USA.
Neurologist. 2010 Jan;16(1):16-22. doi: 10.1097/NRL.0b013e3181b1d5c6.
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 nonfluent 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, whereas 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 multifaceted 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.
额颞叶痴呆最近被认为是青年起病型痴呆的常见病因。
综述当前额颞叶痴呆的临床评估方法、病理生理学认识及治疗。
两种主要临床表现为：（1）行为方面，表现为冲动行为和去抑制、人格改变如淡漠和冷漠以及判断力差；（2）语言方面，表现为非流利性失语伴命名障碍（原发性进行性失语），或流利性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、影响大脑的血管性疾病和其他疾病以及精神疾病。包括神经心理学测试以及脑结构和功能成像在内的检查可能有助于支持诊断。在病理生理学认识方面的最新进展表明，大多数病例存在潜在的泛素阳性包涵体，而有些病例存在tau阳性包涵体。已鉴定出基因突变，特别是tau或原颗粒蛋白基因的17号染色体突变。治疗包括试用对症药物以及多方面方法，包括环境调整和长期护理规划。
研究额颞叶痴呆的医学研究人员旨在确定疾病修饰药物，并最终找到治愈这种毁灭性疾病的方法。
