DOI: 10.1007/s13311-016-0484-9 Pages: 11-23
Article Type: Review

Neuroimaging Endpoints in Amyotrophic Lateral Sclerosis

1. University of Oxford, Nuffield Department of Clinical Neurosciences

2. Vita-Salute San Raffaele University, Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute

3. Jena University Hospital, Hans-Berger Department of Neurology

4. Vita-Salute San Raffaele University, Department of Neurology, San Raffaele Scientific Institute

Correspondence to:
Martin R. Turner
Email: martin.turner@ndcn.ox.ac.uk

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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.

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  • Online: Oct 17, 2016

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