伴钙化的弥散性神经原纤维缠结病

Diffuse neurofibrillary tangles with caleification

伴钙化的弥散性神经原纤维缠结病(DNTC)是罕见的神经系统退行性疾病, 属Tau蛋白病范畴。DNTC的主要特征为老年期前痴呆、双侧基底节和小脑齿状核对称性钙化以及双侧颞叶萎缩。目前, 已报道的DNTC病例绝大多数来自日本, 个例来自其他国家。虽然DNTC是基于尸检的病理诊断, 但结合临床特点及正电子发射计算机断层成像技术对脑内tau蛋白的显像, 使DNTC可被生前诊断。本文对DNTC的临床特点、病理改变及诊断标准等进行综述。

Diffuse neurfibrllary tangles with caleification(DNTC)is a rare neurodegenerative disease and belongs to the category of tauopathies.The main characteristics of DNTC include presenile dementia.symmetrical bilateral caleification in the basal ganglia and cerebral dentate nuclei.and bilateral atrophy of the temporal lobes.At present.the majority of reported cases with DNTC were from Japan.with only a few cases from other countries.Although DNTC is a pathological diagnosis based on autopsy.ante mortem diagnosis for DNTC can be achieved based on clinical features and presentation of brain tau protein shown on positron emission computed tomography.The clinical characteristics.pathological changes and diagnosis criteria of DNTC are reviewed in this paper.