Litvinov V V, Freynd G G
Perm State Medical University named after academician E.A. Wagner, Perm, Russia.
Arkh Patol. 2024;86(4):51-57. doi: 10.17116/patol20248604151.
Diseases morphologically characterized by frontotemporal lobar degeneration have relatively recently been considered as a group of frontotemporal dementias. This group is characterized by a tendency to early clinical onset of dementia, common genetic and morphological features, as well as a possible association with diseases such as amyotrophic lateral sclerosis and atypical parkinsonism syndrome. Historically, Pick's dementia (Pick's disease) was described as the first of the frontotemporal dementias, which is morphologically characterized by the presence of argyrophilic Pick's bodies represented by 3R-tau protein in the neurons of the cerebral cortex. Despite the characteristic clinical and morphological picture due to the relative rarity, the diagnosis of Pick's dementia is infrequently made by both clinicians and pathologists. The article presents current data on frontotemporal dementia. A case of Pick's dementia with characteristic clinical manifestations in the form of early onset of behavioral and personality disorders, as well as specific morphological changes in the brain, is described.
形态学上以额颞叶变性为特征的疾病，直到最近才被视为一组额颞叶痴呆症。该组疾病的特点是痴呆症临床发病较早、具有共同的遗传和形态学特征，以及可能与肌萎缩侧索硬化症和非典型帕金森综合征等疾病相关。从历史上看，皮克氏痴呆症（皮克氏病）被描述为最早的额颞叶痴呆症，其形态学特征是在大脑皮质神经元中存在由3R- tau蛋白代表的嗜银性皮克氏小体。尽管由于相对罕见而具有特征性临床和形态学表现，但临床医生和病理学家都很少做出皮克氏痴呆症的诊断。本文介绍了有关额颞叶痴呆症的当前数据。描述了一例皮克氏痴呆症病例，其具有行为和人格障碍早发形式的特征性临床表现以及大脑中的特定形态学变化。
