Abstract

Background:

The frontal variant of Alzheimer’s disease (fAD) is poorly understood and poorly defined. The diagnosis remains challenging. The main differential diagnosis is the behavioral variant of frontotemporal degeneration (bvFTD). For fAD, there is some dissociation between the clinical frontal presentation and imaging and neuropathological studies, which do not always find a specific involvement of the frontal lobes. DAPHNE is a behavioral scale, which demonstrated excellent performance to distinguish between bvFTD and AD.

Objective:

The aim of the present study was to assess the reliability of this new tool to improve the clinical diagnosis of fAD.

Methods:

Twenty fAD patients and their caregivers were prospectively included and were compared with 36 bvFTD and 22 AD patients.

Results:

The three main behavioral disorders in the fAD patients were apathy, loss of empathy, and disinhibition. Three disorders were discriminant because they were less frequent and less severe in the fAD patients than in the bvFTD patients, namely hyperorality, neglect, and perseverations. This specific pattern of behavioral disorders was corroborated by SPECT or ¹⁸FDG PET-CT scan that showed that patients with fAD could have a medial frontal hypoperfusion, whereas in bvFTD patients the orbitofrontal cortex was the main involved region, with more diffuse hypoperfusion.

Conclusion:

We demonstrated that DAPHNE had good sensitivity and good specificity to discriminate between the three groups and in particular between fAD and bvFTD patients. DAPHNE is a quick tool that could help clinicians in memory clinics not only to differentiate bvFTD from typical AD but also from fAD.

中文翻译：

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DAPHNE量表改善阿尔茨海默病额叶变异型的诊断

摘要

背景：

阿尔茨海默病 (fAD) 的额叶变异知之甚少且定义不清。诊断仍然具有挑战性。主要的鉴别诊断是额颞叶变性（bvFTD）的行为变异。对于 fAD，临床额叶表现与影像学和神经病理学研究之间存在一些分离，这些研究并不总是发现特定的额叶受累。DAPHNE 是一种行为量表，在区分 bvFTD 和 AD 方面表现出优异的性能。

客观的：

本研究的目的是评估这种新工具的可靠性，以改善 fAD 的临床诊断。

方法：

20 名 fAD 患者及其护理人员被前瞻性纳入，并与 36 名 bvFTD 和 22 名 AD 患者进行了比较。

结果：

fAD 患者的三种主要行为障碍是冷漠、同理心丧失和去抑制。三种疾病是可区分的，因为它们在 fAD 患者中的频率和严重程度低于 bvFTD 患者，即口齿不清、忽视和坚持。SPECT 或¹⁸ FDG PET-CT 扫描证实了这种特定的行为障碍模式，表明 fAD 患者可能存在内侧额叶灌注不足，而在 bvFTD 患者中，眶额皮质是主要受累区域，具有更多的弥漫性灌注不足。

结论：

我们证明 DAPHNE 在区分三组尤其是 fAD 和 bvFTD 患者方面具有良好的敏感性和特异性。DAPHNE 是一种快速工具，可以帮助记忆诊所的临床医生不仅将 bvFTD 与典型 AD 区分开来，还可以与 fAD 区分开来。