Abstract
Amyotrophic lateral sclerosis (ALS) is the most common adult onset motor neuron disease with no effective disease modifying therapies at present. Spinal cord degeneration is a hallmark feature of ALS, highlighted in the earliest descriptions of the disease by Lockhart Clarke and Jean-Martin Charcot. The anterior horns and corticospinal tracts are invariably affected in ALS, but up to recently it has been notoriously challenging to detect and characterize spinal pathology in vivo. With recent technological advances, spinal imaging now offers unique opportunities to appraise lower motor neuron degeneration, sensory involvement, metabolic alterations, and interneuron pathology in ALS. Quantitative spinal imaging in ALS has now been used in cross-sectional and longitudinal study designs, applied to presymptomatic mutation carriers, and utilized in machine learning applications. Despite its enormous clinical and academic potential, a number of physiological, technological, and methodological challenges limit the routine use of computational spinal imaging in ALS. In this review, we provide a comprehensive overview of emerging spinal cord imaging methods and discuss their advantages, drawbacks, and biomarker potential in clinical applications, clinical trial settings, monitoring, and prognostic roles.
Highlights
Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive neurodegenerative disorder
Due to the plethora of methodological challenges, such as the small cross-sectional area of the human spinal cord, respiratory, and cardiac movement effects, the overwhelming majority of imaging studies have focused on cerebral alterations in ALS [4]
Spinal Magnetic resonance imaging (MRI) parameters are more predictive of survival than clinical variables (ALSFRS-R, MMT, and disease duration) Spinal gray matter metrics are more sensitive to discriminate ALS patients from controls than overall cord cross-sectional area (CSA)
Summary
Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive neurodegenerative disorder. Due to the plethora of methodological challenges, such as the small cross-sectional area of the human spinal cord, respiratory, and cardiac movement effects, the overwhelming majority of imaging studies have focused on cerebral alterations in ALS [4]. More specific gray and white matter measures can be derived from higher resolution images followed by tissue-type segmentation methods [72, 76, 77] Novel quantitative approaches, such as tensor based morphometry and surface based-morphometry permit a more fine-grained characterization of cord topography and the definition of diseaseassociated signatures [74, 78]. Macromolecular spins can be saturated using an off-resonance RF pulse, the magnetization transfer between bound and free pools
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