Wong Stephanie, Strudwick Jessica, Devenney Emma, Hodges John R, Piguet Olivier, Kumfor Fiona
a Brain and Mind Centre , The University of Sydney , Sydney , Australia.
b School of Psychology , The University of Sydney , Sydney , Australia.
Neurocase. 2019 Feb-Apr;25(1-2):48-58. doi: 10.1080/13554794.2019.1609523. Epub 2019 May 2.
The current clinical diagnostic criteria for Alzheimer's disease (AD) recognize an atypical, non-amnestic presentation of AD, characterized by prominent executive dysfunction. Increasing evidence, however, indicates that the clinical phenotype of this so-called "frontal-variant" of AD (fv-AD) includes behavioral symptoms and deficits in social cognition, together with disproportionate frontal lobe atrophy. As these features resemble those characteristic of behavioral-variant frontotemporal dementia (bvFTD), differential diagnosis can be challenging. Here, we report a case of fv-AD who met clinical diagnostic criteria bvFTD, but had in vivo amyloid neuroimaging evidence of AD pathology. We compare this case against two individuals who were clinically diagnosed with bvFTD and early-onset AD, with in vivo amyloid neuroimaging confirmation of pathology. We highlight the challenges in differential diagnosis by contrasting their behavioral, cognitive and structural neuroimaging findings.
目前阿尔茨海默病(AD)的临床诊断标准认可AD的一种非典型、非遗忘型表现,其特征为显著的执行功能障碍。然而,越来越多的证据表明,这种所谓的AD“额叶变异型”(fv-AD)的临床表型包括行为症状和社会认知缺陷,以及不成比例的额叶萎缩。由于这些特征与行为变异型额颞叶痴呆(bvFTD)的特征相似,鉴别诊断可能具有挑战性。在此,我们报告一例符合bvFTD临床诊断标准但有AD病理学活体淀粉样神经影像学证据的fv-AD病例。我们将该病例与另外两名临床诊断为bvFTD和早发性AD且有病理学活体淀粉样神经影像学确认的个体进行比较。我们通过对比他们的行为、认知和结构神经影像学结果,突出了鉴别诊断中的挑战。