Introduction

Abnormal behavioural and physiological reactivity to emotional stimuli is a hallmark of frontotemporal dementia (FTD), particularly the behavioural variant (bvFTD). As part of this repertoire, altered phobic responses have been reported in some patients with FTD but are poorly characterised.

Methods

We collected data (based on caregiver reports) concerning the prevalence and nature of any behavioural changes related to specific phobias in a cohort of patients representing canonical syndromes of FTD and Alzheimer's disease (AD), relative to healthy older controls. Neuroanatomical correlates of altered phobic reactivity were assessed using voxel-based morphometry.

Results

46 patients with bvFTD, 20 with semantic variant primary progressive aphasia, 25 with non-fluent variant primary progressive aphasia, 29 with AD and 55 healthy age-matched individuals participated. Changes in specific phobia were significantly more prevalent in the combined FTD cohort (15.4% of cases) and in the bvFTD group (17.4%) compared both to healthy controls (3.6%) and patients with AD (3.5%). Attenuation of phobic reactivity was reported for individuals in all participant groups, however new phobias developed only in the FTD cohort. Altered phobic reactivity was significantly associated with relative preservation of grey matter in left posterior middle temporal gyrus, right temporo-occipital junction and right anterior cingulate gyrus, brain regions previously implicated in contextual decoding, salience processing and reward valuation.

Conclusion

Altered phobic reactivity is a relatively common issue in patients with FTD, particularly bvFTD. This novel paradigm of strong fear experience has broad implications: clinically, for diagnosis and patient well-being; and neurobiologically, for our understanding of the pathophysiology of aversive sensory signal processing in FTD and the neural mechanisms of fear more generally.

中文翻译：

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额颞叶痴呆的恐惧反应改变：行为和神经解剖学分析。

介绍

对情绪刺激的异常行为和生理反应是额颞叶痴呆 (FTD) 的标志，特别是行为变异 (bvFTD)。作为该曲目的一部分，在一些 FTD 患者中报告了改变的恐惧反应，但特征很差。

方法

我们收集了数据（基于护理人员报告），这些数据与健康的老年对照组相比，在一组代表典型 FTD 和阿尔茨海默病 (AD) 综合征的患者中，与特定恐惧症相关的任何行为变化的发生率和性质。使用基于体素的形态测量法评估改变的恐惧反应的神经解剖学相关性。

结果

46 名 bvFTD 患者、20 名语义变异原发性进行性失语症患者、25 名非流利变异原发性进行性失语症患者、29 名 AD 患者和 55 名健康年龄匹配的个体参与。与健康对照组（3.6%）和 AD 患者（3.5%）相比，特定恐惧症的变化在 FTD 联合组（15.4% 的病例）和 bvFTD 组（17.4%）中更为普遍。据报道，所有参与者组中的个体恐惧反应减弱，但仅在 FTD 队列中出现新的恐惧症。改变的恐惧反应与左后颞中回、右颞枕交界处和右前扣带回的灰质相对保留显着相关，这些大脑区域以前涉及上下文解码、显着性处理和奖励评估。

结论

恐惧反应改变是 FTD 患者相对常见的问题，尤其是 bvFTD。这种强烈恐惧体验的新范式具有广泛的意义：临床上，用于诊断和患者福祉；在神经生物学上，我们对 FTD 中厌恶性感觉信号处理的病理生理学和更普遍的恐惧神经机制的理解。