IMR Press / JIN / Volume 21 / Issue 3 / DOI: 10.31083/j.jin2103087
Open Access Review
Neurometabolic Alterations in Motor Neuron Disease: Insights from Magnetic Resonance Spectroscopy
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1 Medical Physics Laboratory, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece
2 First Department of Neurology, Aeginition Hospital, School of Medicine, National and Kapodistrian University of Athens, 11522 Athens, Greece
3 Second Department of Psychiatry, General University Hospital “Attikon”, School of Medicine, National and Kapodistrian University of Athens, 12462 Athens, Greece
4 Second Department of Radiology, General University Hospital “Attikon”, School of Medicine, National and Kapodistrian University of Athens, 11522 Athens, Greece
5 Computational Neuroimaging Group, Trinity Biomedical Sciences Institute, Trinity College Dublin, D02 VR66 Dublin, Ireland
6 Biomedical Imaging Laboratory (LIB), Pitié-Salpêtrière University Hospital, Sorbonne University, 75005 Paris, France
*Correspondence: christidi.f.a@gmail.com (Foteini Christidi)
These authors contributed equally as joint first-authors.
§Joint senior-authors.
Academic Editor: Luis Puelles
J. Integr. Neurosci. 2022, 21(3), 087; https://doi.org/10.31083/j.jin2103087
Submitted: 14 February 2022 | Revised: 30 March 2022 | Accepted: 15 April 2022 | Published: 24 April 2022
(This article belongs to the Special Issue Brain Imaging)
Copyright: © 2022 The Author(s). Published by IMR Press.
This is an open access article under the CC BY 4.0 license.
Abstract

Magnetic resonance spectroscopy (MRS) has contributed important academic insights in motor neuron diseases (MNDs), particularly in amyotrophic lateral sclerosis (ALS). Over the past three decades momentous methodological advances took place, including the emergence of high-field magnetic resonance imaging (MRI) platforms, multi-voxel techniques, whole-brain protocols, novel head-coil designs, and a multitude of open-source imaging suites. Technological advances in MRS are complemented by important conceptual developments in MND, such as the recognition of the importance of extra-motor brain regions, multi-timepoint longitudinal study designs, assessment of asymptomatic mutation carriers, description of genotype-associated signatures, and the gradual characterisation of non-ALS MND phenotypes. We have conducted a systematic review of published MRS studies in MND to identify important emerging research trends, key lessons from pioneering studies, and stereotyped shortcomings. We also sought to highlight notable gaps in the current literature so that research priorities for future studies can be outlined. While MRS remains relatively underutilised in MND compared to other structural, diffusivity and functional imaging modalities, our review suggests that MRS can not only advance our academic understanding of MND biology, but has a multitude of practical benefits for clinical and pharmaceutical trial applications.

Keywords
magnetic resonance spectroscopy
amyotrophic lateral sclerosis
motor neuron disease
biomarkers
clinical trials
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