Batrancourt Bénédicte, Lecouturier Karen, Ferrand-Verdejo Johan, Guillemot Vincent, Azuar Carole, Bendetowicz David, Migliaccio Raffaella, Rametti-Lacroux Armelle, Dubois Bruno, Levy Richard
Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Univ Paris 06, Institut du Cerveau et de la Moelle épiniére (ICM), FRONTlab, Paris, France.
Institut Pasteur, Centre de Bioinformatique, Biostatistique et Biologie Intégrative (C3BI), Paris, France.
Front Neurol. 2019 Aug 28;10:941. doi: 10.3389/fneur.2019.00941. eCollection 2019.
Apathy is one of the six clinical criteria for the behavioral variant of frontotemporal dementia (bvFTD), and it is almost universal in this disease. Although its consequences in everyday life are debilitating, its underlying mechanisms are poorly known, its assessment is biased by subjectivity and its care management is very limited. In this context, we have developed "," a method aimed at providing quantifiable and objective signature(s) of apathy in order to assess it and identify its precise underlying mechanisms. consists of the observation and recording of the patient's behavior when the participant is being submitted to a multiple-phase scenario reproducing a brief real-life situation. It is performed in a functional exploration platform transformed into a fully furnished waiting room equipped with a video and sensor-based data acquisition system. This multimodal method allowed video-based behavior analyses according to predefined behavioral categories (exploration behavior, sustained activities or inactivity) and actigraphy analyses from a 3D accelerometer. The data obtained were also correlated with behavioral/cognitive tests and scales assessing global cognitive efficiency, apathy, cognitive disinhibition, frontal syndrome, depression and anxiety. Here, bvFTD patients ( = 14) were compared to healthy participants ( = 14) during the very first minutes of the scenario, when the participants discovered the room and were encouraged to explore it. We showed that, in the context of facing a new environment, healthy participants first explored it and then engaged in sustained activities. By contrast, bvFTD patients were mostly inactive and eventually explored this new place, but in a more irregular and less efficient mode than normal subjects. This exploration deficit was correlated with apathy, disinhibition and cognitive and behavioral dysexecutive syndromes. These findings led us to discuss the presumed underlying mechanisms responsible for the exploration deficit (an inability to self-initiate actions, to integrate reward valuation and to inhibit involuntary behavior). Altogether, these results pave the way for simple and objective assessment of behavioral changes that represents a critical step for the evaluation of disease progression and efficacy of treatment in bvFTD.
冷漠是行为变异型额颞叶痴呆（bvFTD）的六项临床标准之一，在这种疾病中几乎普遍存在。尽管其在日常生活中的后果令人衰弱，但其潜在机制却鲜为人知，其评估受到主观性的影响，并且其护理管理非常有限。在此背景下，我们开发了一种方法，旨在提供冷漠的可量化和客观特征，以便对其进行评估并确定其确切的潜在机制。该方法包括在参与者面对重现简短现实生活场景的多阶段情景时，观察和记录患者的行为。这在一个功能探索平台上进行，该平台被改造成一个配备齐全的候诊室，配备了基于视频和传感器的数据采集系统。这种多模态方法允许根据预定义的行为类别（探索行为、持续活动或不活动）进行基于视频的行为分析，并通过3D加速度计进行活动记录分析。获得的数据还与评估整体认知效率、冷漠、认知脱抑制、额叶综合征、抑郁和焦虑的行为/认知测试及量表相关。在此，在情景的最初几分钟，当参与者发现房间并被鼓励探索时，将bvFTD患者（n = 14）与健康参与者（n = 14）进行了比较。我们发现，在面对新环境的情况下，健康参与者首先会探索它，然后进行持续活动。相比之下，bvFTD患者大多不活动，最终才探索这个新地方，但方式比正常受试者更不规律且效率更低。这种探索缺陷与冷漠、脱抑制以及认知和行为执行障碍综合征相关。这些发现促使我们讨论导致探索缺陷的假定潜在机制（无法自我发起行动、整合奖励评估以及抑制非自愿行为）。总之，这些结果为行为变化的简单客观评估铺平了道路，这是评估bvFTD疾病进展和治疗效果的关键一步。
