Department of Advanced Medical and Surgical Sciences, MRI Research Center, Università degli Studi della Campania "Luigi Vanvitelli", P.Zza Miraglia 2, 80138, Naples, Italy.
Brain Imaging Behav. 2023 Feb;17(1):77-89. doi: 10.1007/s11682-022-00744-4. Epub 2022 Nov 12.
Pseudobulbar affect (PBA), referring to exaggerated or inappropriate episodes of laughing and/or crying without an apparent motivating stimulus, has been mainly attributed to bilateral degeneration of corticobulbar tracts. We aimed at exploring brain functional connectivity (FC) correlates of PBA in patients with amyotrophic lateral sclerosis (ALS), the most common motor neuron disease, frequently associated with PBA. Resting state functional MRI (RS-fMRI) independent component (ICA) and seed-based analyses and voxel-based morphometry (VBM) whole-brain analysis were performed on 27 ALS patients (13 with PBA; 14 without PBA) and 26 healthy controls (HC), for investigating functional and structural abnormalities in ALS patients compared to HC and in patients with PBA compared to patients without PBA. Between-patient analysis revealed different FC patterns, especially regarding decreased FC in several areas of cognitive (default mode, frontoparietal, salience) and sensory-motor networks in patients with PBA compared to those without PBA. However, no significant differences were found in gray matter atrophy. Seed-based analysis showed increased FC between middle cerebellar peduncles and posterior cingulate cortex and decreased FC between middle cerebellar peduncles and left middle frontal gyrus in patients with PBA compared to patients without PBA. Our findings suggest that some alterations of fronto-tempo-parietal-cerebellar circuits could be related to PBA in ALS. In particular, the abnormal FC between cerebellum and posterior cingulate cortex and left middle frontal gyrus in patients with PBA compared to patients without PBA highlights a crucial role of the cerebellum in regulating emotion expression in patients with ALS.
假性延髓情绪障碍（PBA）是指在没有明显刺激的情况下出现夸张或不适当的大笑和/或哭泣，主要归因于皮质延髓束的双侧变性。我们旨在探索肌萎缩侧索硬化症（ALS）患者（最常见的运动神经元疾病，常伴有 PBA）的 PBA 的脑功能连接（FC）相关性。对 27 例 ALS 患者（13 例伴有 PBA；14 例无 PBA）和 26 例健康对照（HC）进行了静息态功能磁共振成像（RS-fMRI）独立成分（ICA）和种子分析以及基于体素的形态计量学（VBM）全脑分析，以研究 ALS 患者与 HC 相比和 PBA 患者与无 PBA 患者相比的功能和结构异常。患者间分析显示出不同的 FC 模式，尤其是在伴有 PBA 的患者中，与无 PBA 的患者相比，认知（默认模式、额顶叶、突显）和感觉运动网络的几个区域的 FC 降低。然而，未发现灰质萎缩的显著差异。种子分析显示，与无 PBA 的患者相比，伴有 PBA 的患者的中脑小脑脚和后扣带回之间的 FC 增加，而中脑小脑脚和左侧额中回之间的 FC 减少。我们的研究结果表明，额颞顶-小脑回路的一些改变可能与 ALS 中的 PBA 有关。特别是与无 PBA 的患者相比，伴有 PBA 的患者小脑与后扣带回和左侧额中回之间的异常 FC 突出了小脑在调节 ALS 患者情绪表达中的关键作用。
