痴呆是两种独立的疾病还是突触核蛋白病临床谱中的一种临床实体?

E. Papuć
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摘要

摘要导读:路易体痴呆(DLB)和帕金森病痴呆(PDD)都是重要的痴呆综合征,它们在临床特征、病程、神经病理异常以及治疗方法上都有重叠。然而,目前尚不清楚DLB和PDD是需要区分的两种不同疾病,还是路易体病谱系中的一种临床实体。目前,这些疾病主要是根据痴呆和帕金森症状发病的相对时间来区分的。本文介绍了目前关于这两种疾病的发病机制及其可能重叠的概念。材料和方法:检索DLB和PDD领域的在线数据库,寻找可能符合条件的文章。只选择2000年以后发表的最新文章。结果:DLB与PDD的临床特征相似,包括痴呆伴幻觉和认知波动,以及帕金森症状。PDD和DLB的认知缺陷也相似,以执行功能障碍、视觉空间缺陷和记忆障碍为主。这两种疾病的神经病理改变均涉及脑干、边缘和新皮层中路易小体和路易神经突的存在,以及中脑多巴胺细胞的丢失和前脑腹侧核胆碱能神经元的丢失。结论:DLB和PDD在临床表现、神经心理缺陷和神经病理异常上的相似性可能提示两者是同一疾病的两种不同表型。这篇综述文章介绍了目前关于这两种临床实体之间的异同的知识,并提出了他们是否需要区分的问题。
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Dementia with Lewy bodies and Parkinson’s disease dementia-two independent disorders or one clinical entity within a clinical spectrum of synucleinopathies?
Abstract Introduction: Introduction: Both dementia with Lewy bodies (DLB) and Parkinson’s disease dementia (PDD) are important dementia syndromes that overlap in their clinical features and clinical course, neuropathological abnormalities, and also therapeutic approach. Nevertheless it is still unclear whether DLB and PDD are two different disorders that require differentiation or are one clinical entity within a spectrum of Lewy body disease. Currently these disorders are mainly distinguished on the basis of the relative timing of the onset of symptoms of dementia and parkinsonism. The present paper presents current concepts on the pathogenesis of both disorders and their possible overlap. Material and methods: Online databases in the field of DLB and PDD were searched for to find potentially eligible articles. Only most recent articles published after the year 2000 were chosen. Results: The clinical features of DLB and PDD are similar and include dementia with hallucinations and cognitive fluctuations, as well as parkinsonian signs. Also cognitive deficits are similar in PDD and in DLB, with predominance of executive dysfunction, visual-spatial deficits and memory impairment. Neuropathological changes in both disorders involve the presence of Lewy bodies and Lewy neurites within brainstem, limbic and neocortex, as well as loss of midbrain dopamine cells, and loss of cholinergic neurons in the nuclei of ventral forebrain. Conclusions: Similarities in clinical manifestation, neuropsychological deficits and neuropathological abnormalities may suggest that both DLB and PDD are two different phenotypes of the same disorder. This review article presents current knowledge on similarities and differences between these two clinical entities and raises the question whether they require differentiation or not.
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期刊介绍: The quarterly Current Problems of Psychiatry is a continuation of the volume "Research on Schizophrenia" and is addressed to a wide group of psychiatrists and clinical psychologists. The quarterly is a reviewed scientific journal of international scope, publishing original papers, review papers, case studies, conference reports, letters to the editor and book reviews. The aim of the "Current Problems of Psychiatry" is providing a wide audience with scientific works, representing a significant contribution to the development of psychiatry and clinical psychology. The works published in the journal are printed in Polish and English. Terms and Conditions for publishing manuscripts in the quarterly are available on the website www.cppsych.umlub.pl in the tab "Requirements".
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