Diagnosing the frontal variant of Alzheimer's disease: a clinician's yellow brick road.

Journal of Clinical Movement Disorders Pub Date : 2017-03-02 eCollection Date: 2017-01-01 DOI:10.1186/s40734-017-0052-4
Russell P Sawyer, Federico Rodriguez-Porcel, Matthew Hagen, Rhonna Shatz, Alberto J Espay
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引用次数: 30

Abstract

Background: Disruption of the frontal lobes and its associated networks are a common consequence of neurodegenerative disorders. Given the wide range of cognitive, behavioral and motor processes in which the frontal lobes are involved, there can be a great variety of manifestations depending on the pathology distribution. The most common are the behavioral variant of frontotemporal dementia (bvFTD) and the frontal variant of Alzheimer's disease (fvAD), which are particularly challenging to disentangle. Recognizing fvAD from bvFTD-related pathologies is a diagnostic challenge and a critical need in the management and counseling of these patients.

Case presentation: Here we present three pathology-proven cases of Alzheimer's disease initially misdiagnosed as bvFTD and discuss the distinctive or less overlapping historical, examination, and laboratory findings of fvAD and bvFTD, deriving analogies for mnemonic endurance from the Wizard of Oz worldview.

Conclusion: The Yellow Brick Road to diagnosing these disorders may be served by the metaphor of fvAD as the irritable, paranoid, and tremulous Scarecrow and bvFTD the heartless, ritualistic, and rigid Tin Man. An Oz-inspired creative license may help the clinician recognize the differential disease progression, caregiver burden, and treatment response of fvAD compared with bvFTD.

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诊断阿尔茨海默病的额叶变体:临床医生的黄砖路。
背景:额叶及其相关网络的破坏是神经退行性疾病的常见后果。由于额叶参与了广泛的认知、行为和运动过程,因此根据病理分布的不同,可以有多种表现。最常见的是额颞叶痴呆(bvFTD)的行为变异和阿尔茨海默病(fvAD)的额叶变异,这两种疾病的研究尤其具有挑战性。从bvftd相关病理中识别fvAD是一项诊断挑战,也是这些患者管理和咨询的关键需求。病例介绍:在这里,我们提出了三例经病理证实的阿尔茨海默病,最初被误诊为bvFTD,并讨论了fvAD和bvFTD的独特或较少重叠的历史,检查和实验室结果,从绿野仙踪的世界观中得出记忆耐力的类比。结论:将fvAD比喻为易怒、偏执、颤抖的稻草人,将bvFTD比喻为无情、仪式、刻板的铁皮人,可以作为诊断这些障碍的“黄砖路”。oz启发的创造性许可可以帮助临床医生认识到与bvFTD相比,fvAD的不同疾病进展、照顾者负担和治疗反应。
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