Kovacs G G
Institute of Neurology, Medical University of Vienna, Vienna, Austria.
Neuropathol Appl Neurobiol. 2015 Feb;41(1):3-23. doi: 10.1111/nan.12208.
Tauopathies are clinically, morphologically and biochemically heterogeneous neurodegenerative diseases characterized by the deposition of abnormal tau protein in the brain. The neuropathological phenotypes are distinguished based on the involvement of different anatomical areas, cell types and presence of distinct isoforms of tau in the pathological deposits. The nomenclature of primary tauopathies overlaps with the modern classification of frontotemporal lobar degeneration. Neuropathological phenotypes comprise Pick's disease, progressive supranuclear palsy, corticobasal degeneration, argyrophilic grain disease, primary age-related tauopathy, formerly called also as neurofibrillary tangle-only dementia, and a recently characterized entity called globular glial tauopathy. Mutations in the gene encoding the microtubule-associated protein tau are associated with frontotemporal dementia and parkinsonism linked to chromosome 17. In addition, further neurodegenerative conditions with diverse aetiologies may be associated with tau pathologies. Thus, the spectrum of tau pathologies and tauopathy entities expands beyond the traditionally discussed disease forms. Detailed multidisciplinary studies are still required to understand their significance.
tau蛋白病是一类在临床、形态学和生物化学方面具有异质性的神经退行性疾病，其特征是大脑中异常tau蛋白的沉积。神经病理学表型根据不同解剖区域、细胞类型的受累情况以及病理沉积物中不同tau异构体的存在来区分。原发性tau蛋白病的命名与额颞叶变性的现代分类有重叠。神经病理学表型包括皮克病、进行性核上性麻痹、皮质基底节变性、嗜银颗粒病、原发性年龄相关性tau蛋白病（以前也称为仅神经原纤维缠结性痴呆）以及最近发现的一种称为球状胶质tau蛋白病的实体。编码微管相关蛋白tau的基因突变与17号染色体连锁的额颞叶痴呆和帕金森综合征相关。此外，病因多样的其他神经退行性疾病可能与tau病理改变有关。因此，tau病理改变和tau蛋白病实体的范围超出了传统讨论的疾病形式。仍需要详细的多学科研究来了解它们的意义。
