title: Neuroimaging Endpoints in Amyotrophic Lateral Sclerosis.
abstract: 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.
authors.affiliation: Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
authors.forename: Ricarda A L
authors.initials: RA
authors.lastname: Menke
authors.affiliation: Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Agosta
authors.affiliation: Hans-Berger Department of Neurology, Jena University Hospital, Jena, Germany.
authors.forename: Julian
authors.initials: J
authors.lastname: Grosskreutz
authors.affiliation: Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.|Department of Neurology, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Filippi
authors.affiliation: Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK. martin.turner@ndcn.ox.ac.uk.
authors.forename: Martin R
authors.initials: MR
authors.lastname: Turner
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D000818:Animals; D015415:Biomarkers; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D001931:Brain Mapping; D002986:Clinical Trials as Topic; D056324:Diffusion Tensor Imaging; D004195:Disease Models, Animal; D018450:Disease Progression; D023381:Endpoint Determination; D006801:Humans; D007090:Image Interpretation, Computer-Assisted / Q000379:methods; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D059906:Neuroimaging; D049268:Positron-Emission Tomography
chemical_list: D015415:Biomarkers
keywords: Amyotrophic lateral sclerosis; biomarker.; magnetic resonance imaging; motor neuron disease; trial