连锁于17号染色体伴帕金森病的额颞叶痴呆

Frontotemporal Dementia with Parkinsonism Linked to Chromosome 17

FTIDP-17是一组成年发病的、逐渐进展的神经变性性常染色体显性遗传病。目前认为与tau基因突变密切相关，以过度磷酸化的tau蛋白异常堆积为病理学特征。自1996年确定为一种独立疾病以来，陆续报道了若干家系。现就FFOP-17的发病机制、临床、神经病理学特点等进行综述。

Frontotemporal dementia with parkinsonism linked to chromosome 17（ FTDP-17 ） is an autosomal dominant disorder that presents clinically with dementia, extrapyramidal signs, and behavioral disturbances in mild-life which lead to the accumulation of deposits of hyperphosphorylated tau protein. It is closely related to mutations in the tau gene. Since FTDP-17 was originally defined in the Consensus Conference held in Ann Arbor, Michigan in 1996, it has become apparent that this syndrome has worldwide distribution. So it is important to pay more attention to FTDP-17.