目标:行为变异额颞叶痴呆(bvFTD)和原发性精神疾病(PPD),比如心情,精神病患者,和自闭症谱系障碍,行为和社会认知具有相似的临床特征。更好地了解bvFTD和PPD的临床进展对于充分的疾病监测和试验设计至关重要。
方法:在这项纵向研究中(N=89),接受至少一项随访评估的bvFTD和PPD患者纳入阿姆斯特丹阿尔茨海默病中心社会脑项目.行为改变和社会认知能力下降是通过信息源问卷进行评估的(剑桥行为量表-修订版,正面行为清单[联邦调查局],定型评级清单,额颞叶痴呆评定量表,修订后的自我监测量表[RSMS]-护理人员)和患者评估(Ekman60-Faces测试,RSMS患者,情绪传染量表)。临床轨迹(中位数=1.4年,四分位数间距=1.0-2.2)使用线性混合模型进行检查。在子样本中,检查了与基线血清神经丝光(sNfL)的关联。
结果:在基线时,诊断组之间的行为和社会认知症状相似,除了bvFTD中的情绪识别较差。随着时间的推移,BvFTD的行为症状恶化,而大多数措施保持稳定,FBI在PPD方面有所改善。关于社会认知,在bvFTD中,情绪识别和护理人员报告的社会情绪敏感性恶化,在PPD中保持稳定.患者报告的社会认知指标没有随时间变化。更高的sNfL与更快的行为改变相关。
结论:行为和社会认知的轨迹区分bvFTD和PPD,前提是社会认知不是患者报告的。因此,我们强调需要优化bvFTD中的纵向社会认知评估。sNfL可能是神经精神人群行为进展的有用预后标志物。
OBJECTIVE: Behavioral variant frontotemporal dementia (bvFTD) and primary psychiatric disorders (PPD), such as mood, psychotic, and autism spectrum disorders, share similar clinical characteristics of behavior and social cognition. Better understanding of clinical progression in bvFTD and PPD is essential for adequate disease monitoring and trial design.
METHODS: In this longitudinal study (N = 89), patients with bvFTD and PPD with at least one follow-up assessment were included from the Social Brain Project of the Alzheimer Center Amsterdam. Behavioral change and social cognitive decline were assessed via informant-rated questionnaires (Cambridge Behavioral Inventory-Revised, Frontal Behavioral Inventory [FBI], Stereotypy Rating Inventory, Frontotemporal Dementia Rating Scale, Revised Self-Monitoring Scale [RSMS]-caregiver) and patient assessment (Ekman 60-Faces Test, RSMS-patient, Emotional Contagion Scale). Clinical trajectories (median = 1.4 years, interquartile range = 1.0-2.2) were examined using linear mixed models. In a subsample, associations with baseline serum neurofilament light (sNfL) were examined.
RESULTS: At baseline, behavioral and social cognitive symptoms were similar between diagnosis groups, except for poorer emotion recognition in bvFTD. Over time, behavioral symptoms worsened in bvFTD, whereas most measures remained stable and the FBI improved in PPD. Regarding social cognition, emotion recognition and caregiver-reported socioemotional sensitivity worsened in bvFTD and remained stable in PPD. Patient-reported social cognitive measures did not change over time. Higher sNfL was associated with faster behavioral change.
CONCLUSIONS: Trajectories of behavior and social cognition differentiate bvFTD from PPD, provided that social cognition is not patient-reported. Therefore, we stress the need to optimize longitudinal social cognitive assessment in bvFTD. sNfL may be a useful prognostic marker of behavioral progression in neuropsychiatric populations.