Distribution and characteristics of transactive response DNA binding protein 43 kDa pathology in progressive supranuclear palsy
Distribution and characteristics of transactive response DNA binding protein 43 kDa pathology in progressive supranuclear palsy
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DOI：
10.1002/mds.26809
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发表时间：
2017-02-01
影响因子：
8.6
通讯作者：
Dickson, Dennis W.
中科院分区：
文献类型：
--
作者：
Koga, Shunsuke;Sanchez-Contreras, Monica;Dickson, Dennis W.
背景本研究的目的是确定PSP中的反式反应DNA结合蛋白43 kDa病理的频率、具有这种病理的患者的临床特征以及其遗传危险因素。在杏仁核中筛选海马体阴性的261例病例的子集。这种病理的密度和破坏，以及区域tau蛋白负荷，临床和遗传特征进行了analysed.ResultsWe观察到的反式反应DNA结合蛋白43 kDa的病理在47例海马和另外9例只影响杏仁核。海马硬化是最强的危险因素，其次是阿尔茨海默病病理学，嗜银颗粒病和老年死亡。PSP的DNA结合蛋白43 kDa病理分为5个阶段：A期仅在杏仁核（16%），I期仅在海马和内嗅皮质（9%），II期包括A期和I期（38%），III期进一步扩散到内侧枕颞回（20%），III期仅在杏仁核（16%），I期仅在海马和内嗅皮质（9%）。IV期额叶背外侧病变占18%。易受PSP病理影响的解剖区域在II期及以后有不同程度的病理改变。PSP与transactive response DNA结合蛋白43 kDa的病理年龄较大，在疾病发作时，有较低的中位数MMSE评分，但是，后者是由concurrent pathologies.ConclusionsDistribution和临床特点的transactive response DNA结合蛋白43 kDa的病理在PSP的影响，并发的病理。这是第一项观察到PSP脆弱区域也易受这种非tau病理学影响的研究。(c)2016国际帕金森和运动障碍协会。
BackgroundThis study aimed to determine the frequency of transactive response DNA binding protein 43 kDa pathology in PSP, the clinical features of patients with this pathology, and genetic risk factors for it.MethodsHippocampal sections were screened with immunohistochemistry for transactive response DNA binding protein 43 kDa in 945 PSP cases. A subset of 261 cases that were negative in hippocampus was screened in the amygdala. The density and disruption of this pathology, as well as regional tau burden, and clinical and genetic characteristics were analyzed.ResultsWe observed transactive response DNA binding protein 43 kDa pathology in 47 cases in the hippocampus and an additional 9 cases that only affected the amygdala. Hippocampal sclerosis was the strongest risk factor, followed by Alzheimer's disease pathology, argyrophilic grain disease, and older age at death. Five stages of transactive response DNA binding protein 43 kDa pathology were identified in PSP: Stage A had pathology only in the amygdala (16%); stage I had pathology confined to the hippocampus and entorhinal cortex (9%); stage II included both regions of stage A and I (38%); stage III spread further to medial occipitotemporal gyrus (20%); and stage IV had pathology in the dorsolateral frontal lobe (18%). Anatomical areas vulnerable to PSP pathology had varying degrees of this pathology in stage II and later. PSP with transactive response DNA binding protein 43 kDa pathology were older at disease onset and had lower median MMSE scores; however, the latter was driven by concurrent pathologies.ConclusionsDistribution and clinical characteristics of transactive response DNA binding protein 43 kDa pathology in PSP were influenced by concurrent pathologies. This is the first study to observe that PSP-vulnerable regions are also susceptible to this non-tau pathology. (c) 2016 International Parkinson and Movement Disorder Society.