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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative, clinically heterogeneous syndrome pathologically overlapping with frontotemporal dementia. To date, therapeutic trials in animal models have not been able to predict treatment response in humans, and the revised ALS Functional Rating Scale, which is based on coarse disability measures, remains the gold-standard measure of disease progression. Advances in neuroimaging have enabled mapping of functional, structural, and molecular aspects of ALS pathology, and these objective measures may be uniquely sensitive to the detection of propagation of pathology in vivo. Abnormalities are detectable before clinical symptoms develop, offering the potential for neuroprotective intervention in familial cases. Although promising neuroimaging biomarker candidates for diagnosis, prognosis, and disease progression have emerged, these have been from the study of necessarily select patient cohorts identified in specialized referral centers. Further multicenter research is now needed to establish their validity as therapeutic outcome measures.

Original publication

DOI

10.1007/s13311-016-0484-9

Type

Journal article

Journal

Neurotherapeutics

Publication Date

01/2017

Volume

14

Pages

11 - 23

Keywords

Amyotrophic lateral sclerosis, biomarker., magnetic resonance imaging, motor neuron disease, trial, Amyotrophic Lateral Sclerosis, Animals, Biomarkers, Brain, Brain Mapping, Clinical Trials as Topic, Diffusion Tensor Imaging, Disease Models, Animal, Disease Progression, Endpoint Determination, Humans, Image Interpretation, Computer-Assisted, Magnetic Resonance Imaging, Magnetic Resonance Spectroscopy, Neuroimaging, Positron-Emission Tomography