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只见树木不见森林?将路易体痴呆的诊断标准应用于表现为后部皮质萎缩的患者。

Seeing the Woods for the Trees? Applying Diagnostic Criteria for Dementia with Lewy Bodies to Patients Presenting with Posterior Cortical Atrophy.

作者信息

Kelly Mark J, Fearon Conor, O'Dowd Seán

机构信息

Tallaght University Hospital.

Academic Unit of Neurology, Trinity College Dublin.

出版信息

Alzheimer Dis Assoc Disord. 2023;37(1):88-91. doi: 10.1097/WAD.0000000000000522. Epub 2022 Aug 4.

Abstract

Posterior cortical atrophy (PCA) is a clinico-radiological syndrome characterised by progressive decline in visual processing and other posterior cognitive functions, relatively preserved memory and language in the early stages, and atrophy of posterior brain regions. Often considered a "visual variant" of Alzheimer's disease, a number of other pathological substrates are recognised. Dementia with Lewy Bodies is the second most common neurodegenerative dementia and there is increasing recognition of presentations with little or no parkinsonism, highlighting significant under-recognition of this condition. To complicate matters, some patients with PCA exhibit additional features consistent with other neurodegenerative conditions. We present a series of three such patients presenting with features satisfying the recent consensus criteria for "PCA-Plus (DLB)". We review the current classification of PCA and highlight the importance of deep clinico-radiological phenotyping in neurodegenerative disease to guide targeted interventions and establish future trial-ready cohorts.

摘要

后部皮质萎缩(PCA)是一种临床放射学综合征,其特征为视觉处理及其他后部认知功能进行性衰退,早期记忆和语言功能相对保留,以及脑后部区域萎缩。PCA常被视为阿尔茨海默病的“视觉变异型”,但也认识到有许多其他病理基础。路易体痴呆是第二常见的神经退行性痴呆,目前越来越多地认识到其临床表现几乎没有或完全没有帕金森症状,这凸显了对该病症的严重认识不足。更复杂的是,一些PCA患者还表现出与其他神经退行性疾病相符的额外特征。我们报告了一系列三名此类患者,他们的表现符合最近关于“PCA加(DLB)”的共识标准。我们回顾了PCA的当前分类,并强调了在神经退行性疾病中进行深入临床放射学表型分析对于指导靶向干预和建立未来适合试验的队列的重要性。

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