Alzheimer Centre Amsterdam, Neurology, Vrije Universiteit Amsterdam, Amsterdam UMC location VUmc, Amsterdam, The Netherlands.
Amsterdam Neuroscience, Neurodegeneration, Amsterdam, The Netherlands.
Psychogeriatrics. 2024 Jul;24(4):741-751. doi: 10.1111/psyg.13107. Epub 2024 Apr 2.
Patients displaying clinical features of behavioural variant of frontotemporal dementia (bvFTD) but lacking both neuroimaging abnormalities and clinical progression are considered to represent the phenocopy syndrome of bvFTD (phFTD). Extensive clinical overlap between early phase bvFTD and phFTD hampers diagnostic distinction. We aimed to assess the diagnostic value of clinician-rated, self-reported and caregiver-reported symptoms for clinical distinction between phFTD and bvFTD.
There were 33 phFTD and 95 probable bvFTD patients included in the study (total N = 128). Clinician-rated, self-reported tests and caregiver-reported symptoms were compared between phFTD and bvFTD on social cognition, behaviour, mood and activities of daily living (ADL). Scores were compared between groups, followed by multiple logistic regression analysis, adjusted for age and sex. Receiver operating characteristic curves were plotted to assess diagnostic value.
Using clinician-rated and self-reported tests, phFTD patients performed better on facial emotion recognition and reported more depressive symptoms. Caregiver-reported behavioural symptoms indicated higher behavioural and ADL impairment in phFTD compared to bvFTD. Facial emotion recognition provided highest diagnostic accuracy for distinction of phFTD from bvFTD (area under the curve (AUC) 0.813 95% CI 0.735-0.892, P < 0.001, sensitivity 81%, specificity 74%) followed by depressive symptoms (AUC 0.769 95% 0.674-0.864, P < 0.001 sensitivity 81%, specificity of 63%).
Social cognition tests are most suitable for distinction of phFTD from bvFTD. Caregiver-reported questionnaires and phFTD diagnosis seemed inversely correlated, showing more symptoms in phFTD. Further research is needed on phFTD aetiology and in caregivers taking into account disease burden to assess what explains this discrepancy between clinician-rated and caregiver-based tools.
表现为行为变异额颞叶痴呆（bvFTD）临床特征但既无神经影像学异常又无临床进展的患者被认为代表了 bvFTD 的表型复制综合征（phFTD）。早期 bvFTD 和 phFTD 之间广泛的临床重叠阻碍了诊断区分。我们旨在评估临床医生评定、自我报告和护理人员报告的症状在 phFTD 和 bvFTD 之间的临床区分中的诊断价值。
研究纳入了 33 例 phFTD 和 95 例可能的 bvFTD 患者（总计 128 例）。在社会认知、行为、情绪和日常生活活动（ADL）方面，比较了 phFTD 和 bvFTD 之间的临床医生评定、自我报告测试和护理人员报告的症状。比较了组间评分，然后进行了多变量逻辑回归分析，调整了年龄和性别。绘制了受试者工作特征曲线以评估诊断价值。
使用临床医生评定和自我报告的测试，phFTD 患者在面部情绪识别方面表现更好，报告的抑郁症状更多。与 bvFTD 相比，护理人员报告的行为症状表明 phFTD 的行为和 ADL 损害更高。面部情绪识别对区分 phFTD 与 bvFTD 的诊断准确性最高（曲线下面积（AUC）0.813 95%置信区间（CI）0.735-0.892，P<0.001，敏感性 81%，特异性 74%），其次是抑郁症状（AUC 0.769 95% CI 0.674-0.864，P<0.001，敏感性 81%，特异性 63%）。
社会认知测试最适合区分 phFTD 与 bvFTD。护理人员报告的问卷和 phFTD 诊断似乎呈负相关，表明 phFTD 的症状更多。需要进一步研究 phFTD 的病因以及照顾者，考虑疾病负担，以评估是什么解释了临床医生评定和基于护理者的工具之间的这种差异。
