Neuroscience Research Australia, Randwick, NSW, Australia.
Lancet Neurol. 2011 Feb;10(2):162-72. doi: 10.1016/S1474-4422(10)70299-4. Epub 2010 Dec 10.
Patients with behavioural-variant frontotemporal dementia (bvFTD) present with insidious changes in personality and interpersonal conduct that indicate progressive disintegration of the neural circuits involved in social cognition, emotion regulation, motivation, and decision making. The underlying pathological changes are heterogeneous and are characterised by various intraneuronal inclusions. Biomarkers to detect these histopathological changes in life are becoming increasingly important with the development of disease-modifying drugs. Gene mutations have been found that collectively account for around 10-20% of cases. Recently, criteria proposed for bvFTD define three levels of diagnostic certainty: possible, probable, and definite. Detailed history taking from family members to elicit behavioural features underpins the diagnostic process, with support from neuropsychological testing designed to detect impairment in decision making, emotion processing, and social cognition. Brain imaging is important for increasing the level of diagnostic certainty. A recently developed staging instrument shows much promise for monitoring patients and evaluating therapies, which at present are aimed at symptom amelioration. Carer education and support remain of paramount importance.
行为变异额颞叶痴呆（bvFTD）患者表现出人格和人际交往行为的逐渐变化，表明涉及社会认知、情绪调节、动机和决策的神经网络逐渐解体。潜在的病理变化具有异质性，其特征是各种神经元内包涵体。随着疾病修饰药物的发展，检测这些组织病理学变化的生物标志物变得越来越重要。已经发现基因突变，这些基因突变总共占 10-20%左右的病例。最近，bvFTD 的诊断标准提出了三个诊断确定性级别：可能、可能和确定。详细的家族史采集以引出行为特征是诊断过程的基础，神经心理学测试支持旨在检测决策、情绪处理和社会认知障碍的测试。脑成像对于提高诊断确定性水平很重要。最近开发的分期工具显示出了监测患者和评估治疗方法的巨大潜力，目前的治疗方法旨在改善症状。照顾者的教育和支持仍然至关重要。
