Division of Neuroradiology, Hospital de Braga, Braga, Portugal.
Department of Epidemiology, National Alzheimer's Coordinating Center, University of Washington, Seattle, Washington, USA.
Acta Neuropathol Commun. 2019 Dec 9;7(1):204. doi: 10.1186/s40478-019-0842-z.
Alzheimer disease (AD) is a neurodegenerative disorder characterized pathologically by the accumulation of amyloid-beta (Aβ) plaques and tau neurofibrillary tangles (NFTs). Recently, primary age-related tauopathy (PART) has been described as a new anatomopathological disorder where NFTs are the main feature in the absence of neuritic plaques. However, since PART has mainly been studied in post-mortem patient brains, not much is known about the clinical or neuroimaging characteristics of PART. Here, we studied the clinical brain imaging characteristics of PART focusing on neuroanatomical vulnerability by applying a previously validated multiregion visual atrophy scale. We analysed 26 cases with confirmed PART with paired clinical magnetic resonance imaging (MRI) acquisitions. In this selected cohort we found that upon correcting for the effect of age, there is increased atrophy in the medial temporal region with increasing Braak staging (r = 0.3937, p = 0.0466). Upon controlling for Braak staging effect, predominantly two regions, anterior temporal (r = 0.3638, p = 0.0677) and medial temporal (r = 0.3836, p = 0.053), show a trend for increased atrophy with increasing age. Moreover, anterior temporal lobe atrophy was associated with decreased semantic memory/language (r = - 0.5823, p = 0.0056; and r = - 0.6371, p = 0.0019, respectively), as was medial temporal lobe atrophy (r = - 0.4445, p = 0.0435). Overall, these findings support that PART is associated with medial temporal lobe atrophy and predominantly affects semantic memory/language. These findings highlight that other factors associated with aging and beyond NFTs could be involved in PART pathophysiology.
阿尔茨海默病(AD)是一种神经退行性疾病,其病理特征为淀粉样β(Aβ)斑块和 tau 神经原纤维缠结(NFTs)的积累。最近,原发性年龄相关性 tau 病(PART)被描述为一种新的解剖病理学疾病,其 NFTs 是主要特征,而没有神经原纤维缠结。然而,由于 PART 主要在死后患者大脑中进行研究,因此对 PART 的临床或神经影像学特征知之甚少。在这里,我们通过应用以前验证过的多区域视觉萎缩量表,研究了 PART 的临床脑成像特征,重点是神经解剖学脆弱性。我们分析了 26 例经证实的 PART 病例,并对其进行了配对的临床磁共振成像(MRI)采集。在这个选定的队列中,我们发现,在纠正年龄影响后,随着 Braak 分期的增加,内侧颞区的萎缩程度增加(r=0.3937,p=0.0466)。在控制 Braak 分期影响后,主要有两个区域,即前颞区(r=0.3638,p=0.0677)和内侧颞区(r=0.3836,p=0.053),随着年龄的增长,萎缩程度呈增加趋势。此外,前颞叶萎缩与语义记忆/语言能力下降有关(r=-0.5823,p=0.0056;r=-0.6371,p=0.0019),内侧颞叶萎缩也是如此(r=-0.4445,p=0.0435)。总的来说,这些发现支持 PART 与内侧颞叶萎缩有关,主要影响语义记忆/语言。这些发现强调了与衰老和 NFTs 相关的其他因素可能参与了 PART 的病理生理学。