From the Department of Pathology and Laboratory Medicine, Upstate Medical University, Syracuse, New York, USA.
Glenn Biggs Institute for Alzheimer's & Neurodegenerative Diseases, University of Texas Health Science Center, San Antonio, Texas, USA.
J Neuropathol Exp Neurol. 2021 Apr 16;80(5):436-445. doi: 10.1093/jnen/nlab032.
Primary age-related tauopathy (PART) is a neurodegenerative entity defined as neurofibrillary degeneration generally restricted to the medial temporal region (Braak stage I-IV) with complete or near absence of diffuse and neuritic plaques. Symptoms range in severity but are generally milder and later in onset than in Alzheimer disease (AD). Recently, an early predilection for neurofibrillary degeneration in the hippocampal CA2 subregion has been demonstrated in PART, whereas AD neuropathologic change (ADNC) typically displays relative sparing of CA2 until later stages. In this study, we utilized a semiquantitative scoring system to evaluate asymmetry of neurofibrillary degeneration between left and right hippocampi in 67 PART cases and 17 ADNC cases. 49% of PART cases demonstrated asymmetric findings in at least one hippocampal subregion, and 79% of the asymmetric cases displayed some degree of CA2 asymmetry. Additionally, 19% of cases revealed a difference in Braak score between the right and left hippocampi. There was a significant difference in CA2 neurofibrillary degeneration (p = 0.0006) and CA2/CA1 ratio (p < 0.0001) when comparing the contralateral sides, but neither right nor left was more consistently affected. These data show the importance of analyzing bilateral hippocampi in the diagnostic evaluation of PART and potentially of other neurodegenerative diseases.
原发性年龄相关性 tau 病 (PART) 是一种神经退行性疾病,其定义为神经纤维缠结通常局限于内侧颞叶区域(Braak 分期 I-IV),弥漫性和神经原纤维缠结完全或几乎不存在。症状的严重程度不一,但通常比阿尔茨海默病 (AD) 更轻且发病更晚。最近,在 PART 中已经证明了海马 CA2 亚区神经纤维缠结的早期倾向,而 AD 神经病理改变 (ADNC) 通常在后期才显示 CA2 的相对保留。在这项研究中,我们利用半定量评分系统评估了 67 例 PART 病例和 17 例 ADNC 病例左右海马之间神经纤维缠结变性的不对称性。至少有一个海马亚区存在不对称发现的 PART 病例占 49%,而不对称病例中有 79%显示出 CA2 一定程度的不对称。此外,19%的病例显示左右海马的 Braak 评分存在差异。比较对侧时,CA2 神经纤维缠结变性(p=0.0006)和 CA2/CA1 比值(p<0.0001)存在显著差异,但右侧和左侧均无更一致的影响。这些数据表明,在 PART 和潜在其他神经退行性疾病的诊断评估中分析双侧海马的重要性。