摘要
进行性核上性麻痹(PSP)是一种tau疾病,主要特征为核上性麻痹、眼肌麻痹、假性延髓麻痹、构音障碍、轴向刚度及痴呆。典型病理包括神经元和神经胶质细胞中的神经元缺失、胶质细胞增生及微管结合蛋白(MAPT)阳性夹杂物,主要在基底神经节、脑干和小脑。PSP的发病机理还没有被完全了解;然而有许多假设。本文综述了关于PSP的目前知识,及线粒体功能障碍、脂质过氧化和基因突变方面的内容。我们也讨论了PSP的临床特征,包括垂直凝视麻痹、假性延髓麻痹、失语症、构音障碍、轴向刚度及神经精神病学症状,如健忘症、过敏性、兴趣丧失和痴呆。就诊断而言,对检测PSP的神经成像颇有兴趣;因此综述了神经成像技术核磁共振成像(MRI)和18F脱氧葡萄糖正电子放射断层造影术。PSP的明确诊断依赖于病理和新的临床挑战的引进,显示已经广泛将其纳入诊断标准。也对PSP的治疗如血清素拮抗剂、α2受体拮抗剂和辅酶Q10进行了讨论。对于PSP的治疗没有有效的方法,所有现有的治疗只是缓解。
关键词: 诊断标准,微管结合tau蛋白,神经成像,进行性核上性麻痹,发病原
Current Medicinal Chemistry
Title:Progressive Supranuclear Palsy: What Do We Know About it?
Volume: 22 Issue: 10
Author(s): Ling Long, Xiao-Dong Cai, Xiao-Bo Wei, Jin-Chi Liao, Yun-Qi Xu, Hui-Min Gao, Xiao-Hong Chen and Qing Wang
Affiliation:
关键词: 诊断标准,微管结合tau蛋白,神经成像,进行性核上性麻痹,发病原
摘要: Progressive supranuclear palsy (PSP) is a progressive tauopathy characterized by supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria, axial rigidity, frontal lobe dysfunction, and dementia. The typical pathology includes neuronal loss, gliosis and microtubule-associated protein tau (MAPT)-positive inclusions in neurons and glial cells, primarily in basal ganglia, brainstem and cerebellum. The pathogenesis of PSP is not yet completely understood; however, there are several hypotheses. This article reviews the present knowledge about PSP, and the concepts underlying mitochondrial dysfunction, lipoperoxidation, and gene mutations. The clinical features of PSP are also discussed; these include vertical gaze palsy, pseudobulbar palsy, aphasia, dysarthria, axial rigidity, and neuropsychiatric symptoms, such as amnesia, irritability, loss of interest, and dementia. In terms of diagnosis, there is considerable interest in neuroimaging for detecting PSP; therefore, neuroimaging techniques such as magnetic resonance imaging (MRI) and [18F]- fluorodeoxyglucose positron-emission tomography (FDG-PET) are reviewed. A definitive diagnosis of PSP depends on pathology, and the introduction of new clinical subtypes challenges presents the widely adopted diagnosis criteria. PSP treatments such as serotonin antagonists, α2 receptor antagonists, and coenzyme Q10 are also discussed. There is no curative therapy for PSP; all of the available treatments are palliative.
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Ling Long, Xiao-Dong Cai, Xiao-Bo Wei, Jin-Chi Liao, Yun-Qi Xu, Hui-Min Gao, Xiao-Hong Chen and Qing Wang , Progressive Supranuclear Palsy: What Do We Know About it?, Current Medicinal Chemistry 2015; 22 (10) . https://dx.doi.org/10.2174/0929867322666150302170552
DOI https://dx.doi.org/10.2174/0929867322666150302170552 |
Print ISSN 0929-8673 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-533X |
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