Journal
FRONTIERS IN NEUROLOGY
Volume 10, Issue -, Pages -Publisher
FRONTIERS MEDIA SA
DOI: 10.3389/fneur.2019.00229
Keywords
motor neuron disease; amyotrophic lateral sclerosis; biomarkers; magnetic resonance imaging; neuroimaging
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Funding
- Andrew Lydon Scholarship
- Health Research Board (HRB-Ireland) [HRB EIA-2017-019]
- Irish Institute of Clinical Neuroscience (IICN)-Novartis Ireland Research Grant
- Iris O'Brien Foundation
- Perrigo Clinician-Scientist Research Fellowship
- Research Motor Neuron (RMN-Ireland) Foundation
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Amyotrophic lateral sclerosis (ALS) encompasses a heterogeneous group of phenotypes with different progression rates, varying degree of extra-motor involvement and divergent progression patterns. The natural history of ALS is increasingly evaluated by large, multi-time point longitudinal studies, many of which now incorporate presymptomatic and post-mortem assessments. These studies not only have the potential to characterize patterns of anatomical propagation, molecular mechanisms of disease spread, but also to identify pragmatic monitoring markers. Sensitive markers of progressive neurodegenerative change are indispensable for clinical trials and individualized patient care. Biofluid markers, neuroimaging indices, electrophysiological markers, rating scales, questionnaires, and other disease-specific instruments have divergent sensitivity profiles. The discussion of candidate monitoring markers in ALS has a dual academic and clinical relevance, and is particularly timely given the increasing number of pharmacological trials. The objective of this paper is to provide a comprehensive and critical review of longitudinal studies in ALS, focusing on the sensitivity profile of established and emerging monitoring markers.
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