Magnetic Resonance Spectroscopy in ALS

Proton magnetic resonance spectroscopy (MRS) provides a means of measuring cerebral metabolites relevant to neurodegeneration in vivo. In amyotrophic lateral sclerosis (ALS), neurochemical changes reflecting neuronal loss or dysfunction (decreased N-actylaspartate [NAA]) is most significant in the motor cortex and corticospinal tracts. Other neurochemical changes observed include increased myo-inositol (mIns), a putative marker of gliosis. MRS confirmation of involvement of non-motor regions such as the frontal lobes, thalamus, basal ganglia, and cingulum are consistent with the multi-system facet of motor neuron disease with ALS being part of a MND-FTD spectrum. MRS-derived markers exhibit an encouraging discriminatory ability to identify patients from healthy controls, however more data is needed to determine its ability to assist with the diagnosis in early stages when upper motor neuron signs are limited, and in distinguishing from disease mimics. Longitudinal change of NAA and mIns do not appear to be reliable in monitoring disease progression. Technological advances in hardware and high field scanning are increasing the number of accessible metabolites available for interrogation.