Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with no effective treatment or cure. ALS is characterized by the death of lower motor neurons (LMNs) in the spinal cord and upper motor neurons (UMNs) in the brain and their networks. Since the lower motor neurons are under the control of UMN and the networks, cortical degeneration may play a vital role in the pathophysiology of ALS. These changes that are not apparent on routine imaging with CT scans or MRI brain can be identified using modalities such as diffusion tensor imaging, functional MRI, arterial spin labelling (ASL), electroencephalogram (EEG), magnetoencephalogram (MEG), functional near-infrared spectroscopy (fNIRS), and positron emission tomography (PET) scan. They can help us generate a representation of brain networks and connectivity that can be visualized and parsed out to characterize and quantify the underlying pathophysiology in ALS. In addition, network analysis using graph measures provides a novel way of understanding the complex network changes occurring in the brain. These have the potential to become biomarker for the diagnosis and treatment of ALS. This article is a systematic review and overview of the various connectivity and network-based studies in ALS.

Highlights

  • Amyotrophic lateral sclerosis has an incidence of around 3/ 100,000

  • Postmortem studies have shown Amyotrophic lateral sclerosis (ALS) to be a degenerative disorder of the anterior horn cells in the spinal cord and the cortical neurons, primarily in the motor cortex. e death of the neuronal cell body leads to degeneration of its axons and tracts leading to progressive disability and death [3,4,5]

  • Frontal functional network between-group differences in disruption associated with amyotrophic lateral sclerosis: an functional near-infrared spectroscopy (fNIRS)-based minimum spanning

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Summary

Introduction

Amyotrophic lateral sclerosis has an incidence of around 3/ 100,000. No definite etiology has been determined even though various genetic and environmental factors have been attributed [1]. ALS is mostly sporadic, but 5–10% of cases can be familial [2]. Postmortem studies have shown ALS to be a degenerative disorder of the anterior horn cells in the spinal cord and the cortical neurons, primarily in the motor cortex. E death of the neuronal cell body leads to degeneration of its axons and tracts leading to progressive disability and death [3,4,5]. In addition to axonal degeneration, there is hyperexcitability of surviving neurons and their networks. Loss of inhibitory neurons, increased glutamatergic activity, and functional reorganization secondary to increased compensation may underlie these changes [5,6,7]. Interhemispheric inhibition is impaired in ALS resulting in mirror movements [8]

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