title: The value of magnetic resonance imaging as a biomarker for amyotrophic lateral sclerosis: a systematic review.
abstract: BACKGROUND
  Amyotrophic lateral sclerosis (ALS) is a fatal, rapidly progressive neurodegenerative disease that mainly affects the motor system. A number of potentially neuroprotective and neurorestorative disease-modifying drugs are currently in clinical development. At present, the evaluation of a drug's clinical efficacy in ALS is based on the ALS Functional Rating Scale Revised, motor tests and survival. However, these endpoints are general, variable and late-stage measures of the ALS disease process and thus require the long-term assessment of large cohorts. Hence, there is a need for more sensitive radiological biomarkers. Various sequences for magnetic resonance imaging (MRI) of the brain and spinal cord have may have value as surrogate biomarkers for use in future clinical trials. Here, we review the MRI findings in ALS, their clinical correlations, and their limitations and potential role as biomarkers.
  
  
  METHODS
  The PubMed database was screened to identify studies using MRI in ALS. We included general MRI studies with a control group and an ALS group and longitudinal studies even if a control group was lacking.
  
  
  RESULTS
  A total of 116 studies were analysed with MRI data and clinical correlations. The most disease-sensitive MRI patterns are in motor regions but the brain is more broadly affected.
  
  
  CONCLUSIONS
  Despite the existing MRI biomarkers, there is a need for large cohorts with long term MRI and clinical follow-up. MRI assessment could be improved by standardized MRI protocols with multicentre studies.
authors: Department of Movement Disorders and Neurology, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.|INSERM U1171, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.
authors: G
authors: http://orcid.org/0000-0002-1291-8703
authors: G
authors: Grolez
authors: Department of Movement Disorders and Neurology, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.|INSERM U1171, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.
authors: C
authors: C
authors: Moreau
authors: Department of Movement Disorders and Neurology, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.|INSERM U1171, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.
authors: V
authors: V
authors: Danel-Brunaud
authors: INSERM U1171, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.|Department of Neuroradiology, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.
authors: C
authors: C
authors: Delmaire
authors: INSERM U1171, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.|Department of Neuroradiology, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.
authors: R
authors: R
authors: Lopes
authors: Laboratoire d'Imagerie Biomédicale, Sorbonne Universités, UPMC Univ Paris 06, CNRS, INSERM, Paris, France.|Département des Maladies du Système Nerveux, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France.
authors: P F
authors: PF
authors: Pradat
authors: Laboratoire d'Imagerie Biomédicale, Sorbonne Universités, UPMC Univ Paris 06, CNRS, INSERM, Paris, France.
authors: M M
authors: MM
authors: El Mendili
authors: Department of Movement Disorders and Neurology, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.|INSERM U1171, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.
authors: L
authors: L
authors: Defebvre
authors: Department of Movement Disorders and Neurology, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France. david.devos@chru-lille.fr.|INSERM U1171, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France. david.devos@chru-lille.fr.|Department of Medical Pharmacology, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France. david.devos@chru-lille.fr.
authors: D
authors: D
authors: Devos
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000000981:diagnostic imaging* / Q000473:pathology; D015415:Biomarkers; D002986:Clinical Trials as Topic; D006801:Humans; D008279:Magnetic Resonance Imaging
chemical_list: D015415:Biomarkers
keywords: Amyotrophic lateral sclerosis; Biomarkers; Diffusion tensor imaging; Magnetic resonance imaging; Magnetic resonance spectroscopy; Morphometry; Spinal cord