Abstract

Purpose: Static and dynamic analyses for identifying functional connectivity (FC) have demonstrated brain dysfunctions in amyotrophic lateral sclerosis (ALS). However, few studies on the stability of dynamic FC have been conducted among ALS patients. This study explored the change of functional stability in ALS and how it correlates with disease severity.Methods: We gathered resting-state functional magnetic resonance data from 20 patients with ALS and 22 healthy controls (HCs). The disease severity was assessed with the Revised ALS Functional Rating Scale (ALSFRS-R). We used a sliding window correlation approach to identify dynamic FC and measured the concordance of dynamic FC over time to obtain the functional stability of each voxel. We assessed the between-group difference in functional stability by voxel-wise two-sample t-test. The correlation between the functional stability index and ALSFRS-R in ALS patients was evaluated using Spearman's correlation analysis.Results: Compared with the HC group, the ALS group had significantly increased functional stability in the left pre-central and post-central gyrus and right temporal pole while decreased functional stability in the right middle and inferior frontal gyrus. The results revealed a significant correlation between ALSFRS-R and the mean functional stability in the right temporal pole (r = −0.452 and P = 0.046) in the ALS patients.Conclusions: ALS patients have abnormal stability of brain functional architecture, which is associated with the severity of the disease.

Highlights

  • Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative motor neuron disorder

  • The subjects included in this investigation comprised 20 patients diagnosed with sporadic ALS and 22 healthy controls (HCs)

  • A set of brain areas with relatively higher values of functional stability were bilaterally observed in the HC group

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Summary

Introduction

Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative motor neuron disorder. Progressive upper and lower motor neuron involvement occurs during the course of the disease [1,2,3]. ALS is considered to be a multisystem disorder with substantial extra-motor involvement, such as in the cognitive system and behavioral system; the etiology of ALS remains unclear. Abnormal Functional Stability in ALS [2,3,4]. The course of ALS progresses rapidly, and most patients suffer respiratory failure and die within 3–5 years of the onset of symptoms [2, 4]. It is important to find new neuroimaging signs that can reveal the pathogenesis of ALS and monitor the disease progress

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