Dysexecutive Alzheimer's Disease with Lewy Body Disease Co-Pathology.

IF 1.8 4区 医学 Q3 CLINICAL NEUROLOGY
Ryan P. Coburn, H. Botha, J. Graff‐Radford, R. Reichard, David T. Jones, V. Ramanan
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Abstract

BACKGROUND Alzheimer's disease can present atypically as a progressive dysexecutive syndrome (dAD), an entity which preferentially affects younger individuals and is frequently misdiagnosed, highlighting the imperative for additional research. OBJECTIVE To characterize the clinical, antemortem neuroimaging, and postmortem neuropathologic features of two cases of young-onset dAD who displayed evidence of Lewy body disease (LBD) co-pathology at autopsy. METHODS Clinical histories, antemortem MRI and PET imaging, and postmortem neuropathologic data were reviewed for each patient. Case Descriptions/Results: Canonical features of dAD were observed in both cases, including progressive and predominant impairment in tasks related to working memory and cognitive flexibility, a lack of major behavioral/personality changes, and evidence of abnormal amyloid and tau deposition by antemortem amyloid and tau PET and postmortem neuropathology. Relative sparing of hippocampal involvement was observed in both individuals, in keeping with many cases of clinically atypical AD. One of the patients developed subtle parkinsonian signs as well as paranoia and irritability in the years prior to passing. In both cases, transitional (brainstem and limbic) LBD co-pathology was observed at autopsy. DISCUSSION Although LBD co-pathology is not uncommon in AD overall, the presence of LBD pathology in these young-onset cases of dAD (including a case with apparent symptomatic correlate) warrants further investigation for broader frequency and underlying pathophysiology. CONCLUSION A better understanding of which specific young-onset AD phenotypes are associated with LBD co-pathology would have important implications for counseling, treatment, clinical trial enrollment, and knowledge on disease mechanisms.
性功能障碍阿尔茨海默病伴路易体病。
背景阿尔茨海默病可以非典型地表现为进行性执行障碍综合征(dAD),这种疾病优先影响年轻人,经常被误诊,这突出了进行进一步研究的必要性。目的描述两例年轻发病的dAD患者的临床、生前神经影像学和尸检神经病理学特征,这些患者在尸检中显示出路易体病(LBD)的共同病理学证据。方法回顾每位患者的临床病史、尸检MRI和PET成像以及尸检神经病理学数据。病例描述/结果:在这两个病例中都观察到了dAD的典型特征,包括与工作记忆和认知灵活性相关的任务的进行性和主要损伤,缺乏主要的行为/性格变化,以及通过生前淀粉样蛋白和tau PET以及死后神经病理学发现的淀粉样蛋白异常和tau沉积的证据。两名患者的海马受累相对较少,这与许多临床非典型AD病例一致。其中一名患者在去世前几年出现了微妙的帕金森综合征以及偏执和易怒。在这两个病例中,尸检时都观察到了移行性(脑干和边缘)LBD的共同病理。讨论尽管LBD合并病理在AD中并不罕见,但在这些年轻发病的dAD病例中存在LBD病理(包括具有明显症状相关性的病例),需要进一步研究更广泛的频率和潜在的病理生理学。结论更好地了解哪些特定的年轻发病AD表型与LBD共同病理学相关,将对咨询、治疗、临床试验登记和疾病机制知识具有重要意义。
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来源期刊
Current Alzheimer research
Current Alzheimer research 医学-神经科学
CiteScore
4.00
自引率
4.80%
发文量
64
审稿时长
4-8 weeks
期刊介绍: Current Alzheimer Research publishes peer-reviewed frontier review, research, drug clinical trial studies and letter articles on all areas of Alzheimer’s disease. This multidisciplinary journal will help in understanding the neurobiology, genetics, pathogenesis, and treatment strategies of Alzheimer’s disease. The journal publishes objective reviews written by experts and leaders actively engaged in research using cellular, molecular, and animal models. The journal also covers original articles on recent research in fast emerging areas of molecular diagnostics, brain imaging, drug development and discovery, and clinical aspects of Alzheimer’s disease. Manuscripts are encouraged that relate to the synergistic mechanism of Alzheimer''s disease with other dementia and neurodegenerative disorders. Book reviews, meeting reports and letters-to-the-editor are also published. The journal is essential reading for researchers, educators and physicians with interest in age-related dementia and Alzheimer’s disease. Current Alzheimer Research provides a comprehensive ''bird''s-eye view'' of the current state of Alzheimer''s research for neuroscientists, clinicians, health science planners, granting, caregivers and families of this devastating disease.
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