摘要虽然阿尔茨海默病的诊断标准已经建立，但临床症状差异很大，其特殊类型额叶变异型与行为变异型额颞叶痴呆在临床症状上非常相似，这为鉴别诊断带来了重大挑战。为此，我们报道1例患者表现为进行性认知障碍，早期显著的执行功能障碍行为异常，磁共振成像显示明显的额颞叶萎缩，初步诊断极易误诊为行为变异型额颞叶痴呆。然而，多模态功能神经影像学结果显示神经病理改变更倾向为可能的额叶变异型阿尔茨海默病。本研究表明，使用详细的神经心理测试、生物学标记物和多模态神经成像来识别这些非典型综合征，将有助于提高诊断和患者管理的准确性。

abstractsAlthough the neuropathologic changes and diagnostic criteria for the neurodegenerative disorder Alzheimer′s diseasehave been established, the clinical symptoms are very different largely.The clinical symptoms of its special type frontal lobe variant and behavioral variant frontotemporal dementia are very similar, which brings great challenges to the differential diagnosis.Therefore, we report a patient with progressive cognitive impairment, early significant executive dysfunction and abnormal behavior, and magnetic resonance imaging showed significant frontotemporal atrophy.It is easy to be misdiagnosed as behavioral variant frontotemporal dementia.However, multimodal functional neuroimaging results show that neuropathological changes are more likely to be frontal variant Alzheimer′s disease.This study shows that the use of detailed neuropsychological tests, biological markers and multimodal neuroimaging to identify these atypical syndromes will help to improve the accuracy of diagnosis and patient management.