额颞痴呆与Pick病

额颞痴呆(frontotemporal dementia,FTD)是以额颞叶萎缩为特征的一组神经变性疾病。病因认为是轴索病变继发神经细胞改变,机制为常染色体显性遗传。病理为双侧额叶,颞叶前端的局限性萎缩,可累及尾状核、壳核、丘脑、黑质等皮质下结构。起病隐袭,早期症状为行为、人格改变,渐进性发展为认知功能损害,迅速进展为语言障碍,晚期通常沉默不语,不能与人交流,可有行为异常。MRI显示额叶、前颞叶萎缩。确诊依赖于病理学证据,如局限性额颞叶萎缩,神经元和神经胶质发现tau蛋白包涵体及Pick小体细胞。目前无特异性治疗的治疗方法。

Frontotemporal dementia and pick disease

Frontotemporal dementia(FTD) was characterized by neurodegenerative diseases of frontotemporal atrophy.Pick disease is a type of FTD.Etiology was caused by nerve cell changes of axonal secondary lesions.Pathogenesis was autosomal dominant inheritance.Pathology lied in the bilateral frontal and front temporal lobe atrophy.It could be extended to the caudate nucleus,putamen,thalamus,substantia nigra and other subcortical structures.Hidden passage of onset and early symptoms were changes of behavior and person...