Temp Anna G M, Dyrba Martin, Büttner Charlotte, Kasper Elisabeth, Machts Judith, Kaufmann Jörn, Vielhaber Stefan, Teipel Stefan, Prudlo Johannes
German Center for Neurodegenerative Diseases (DZNE), Rostock, Germany.
Department of Neurology, Rostock University Medical Center, Rostock, Germany.
Front Neurosci. 2021 Jun 23;15:682100. doi: 10.3389/fnins.2021.682100. eCollection 2021.
Half of all amyotrophic lateral sclerosis-frontotemporal spectrum disorder (ALS-FTSD) patients are classified as cognitively impaired, of which 10% have frontotemporal dementia (FTD), and an additional 40% suffer from a frontotemporal syndrome not severe enough to be described as dementia (cognitively impaired/ALSci). As changes in cerebral function measured by resting-state magnet resonance imaging (rs-fMRI) are known in ALS, we investigated whether group differences in resting-state functional connectivity (RSFC) networks could be observed between ALS patients with different cognitive profiles against healthy controls (HC). Furthermore, we correlated cognition and motor functioning with network connectivity.
Healthy controls, 69, and 97 ALS patients underwent functional MRI scanning and cognitive assessment. The ALS patients were categorized as non-impaired (ALSni; = 68), cognitively impaired (ALSci; = 21), and ALS-FTD ( = 8). Group differences in connectivity of the default mode network (DMN), motor network (MN), and ventral attention network (VAN) were investigated using a full-factorial model; correlations between global cognitive performance, shifting, and motor symptom severity were established using Pearson's correlation.
At a liberal alpha level of uncorrected < 0.005 and a cluster size exceeding 20 voxels, we found widespread decreases in functional connectivity in all three networks when comparing ALS patients to HC. Similar patterns of hypoconnectivity in the bilateral motor cortices and frontotemporal emerged when comparing the ALSci and ALS-FTD patients to those not cognitively impaired. Hyperconnectivity in the DMN temporal gyrus correlated with worse global cognition; moreover, hyperconnectivity in the VAN thalamus, insula, and putamen correlated with worse shifting ability. Better-preserved motor function correlated with higher MN connectivity. Only the motor-related effects prevailed at a more conservative significance level of < 0.001.
Resting-state functional connectivity differs between cognitive profiles of ALS and is directly associated with clinical presentation, specifically with motor function, and cognitive shifting.
所有肌萎缩侧索硬化-额颞叶谱系障碍（ALS-FTSD）患者中有一半被归类为认知障碍，其中10%患有额颞叶痴呆（FTD），另外40%患有程度不足以被描述为痴呆的额颞叶综合征（认知障碍/ALSci）。由于已知ALS患者静息态磁共振成像（rs-fMRI）测量的脑功能变化，我们研究了不同认知特征的ALS患者与健康对照（HC）之间在静息态功能连接（RSFC）网络上是否存在组间差异。此外，我们将认知和运动功能与网络连接性进行了关联。
69名健康对照者和97名ALS患者接受了功能磁共振成像扫描和认知评估。ALS患者被分为无认知障碍（ALSni；n = 68）、认知障碍（ALSci；n = 21）和ALS-FTD（n = 8）。使用全因子模型研究默认模式网络（DMN）、运动网络（MN）和腹侧注意网络（VAN）连接性的组间差异；使用Pearson相关性建立整体认知表现、转换能力和运动症状严重程度之间的相关性。
在未校正的宽松α水平<0.005且簇大小超过20体素时，与HC相比，我们发现所有三个网络的功能连接广泛降低。将ALSci和ALS-FTD患者与无认知障碍患者相比时，双侧运动皮层和额颞叶出现了类似的低连接模式。DMN颞叶回的高连接与较差的整体认知相关；此外，VAN丘脑、岛叶和壳核的高连接与较差的转换能力相关。运动功能保存较好与较高的MN连接性相关。只有与运动相关的效应在更保守的显著性水平<0.001时仍然显著。
ALS的认知特征之间静息态功能连接不同，并且与临床表现直接相关，特别是与运动功能和认知转换相关。
