• Mohamed-Mounir El Mendili, Julien Cohen-Adad, Mélanie Pelegrini-Issac, Serge Rossignol, Régine Morizot-Koutlidis, Véronique Marchand-Pauvert, Caroline Iglesias, Sina Sangari, Rose Katz, Stéphane Lehericy, Habib Benali, and Pierre-François Pradat.
• Sorbonne Universités, UPMC Univ Paris 06, UM CR 2, Laboratoire d'Imagerie Biomédicale, Paris, Île-de-France, France; CNRS, UMR 7371, Laboratoire d'Imagerie Biomédicale, Paris, Île-de-France, France; INSERM, U 1146, Laboratoire d'Imagerie Biomédicale, Paris, Île-de-France, France.
• Plos One. 2014 Jan 1;9(4):e95516.

ObjectiveTo evaluate multimodal MRI of the spinal cord in predicting disease progression and one-year clinical status in amyotrophic lateral sclerosis (ALS) patients.Materials And MethodsAfter a first MRI (MRI1), 29 ALS patients were clinically followed during 12 months; 14/29 patients underwent a second MRI (MRI2) at 11±3 months. Cross-sectional area (CSA) that has been shown to be a marker of lower motor neuron degeneration was measured in cervical and upper thoracic spinal cord from T2-weighted images. Fractional anisotropy (FA), axial/radial/mean diffusivities (λ⊥, λ//, MD) and magnetization transfer ratio (MTR) were measured within the lateral corticospinal tract in the cervical region. Imaging metrics were compared with clinical scales: Revised ALS Functional Rating Scale (ALSFRS-R) and manual muscle testing (MMT) score.ResultsAt MRI1, CSA correlated significantly (P<0.05) with MMT and arm ALSFRS-R scores. FA correlated significantly with leg ALFSRS-R scores. One year after MRI1, CSA predicted (P<0.01) arm ALSFSR-R subscore and FA predicted (P<0.01) leg ALSFRS-R subscore. From MRI1 to MRI2, significant changes (P<0.01) were detected for CSA and MTR. CSA rate of change (i.e. atrophy) highly correlated (P<0.01) with arm ALSFRS-R and arm MMT subscores rate of change.ConclusionAtrophy and DTI metrics predicted ALS disease progression. Cord atrophy was a better biomarker of disease progression than diffusion and MTR. Our study suggests that multimodal MRI could provide surrogate markers of ALS that may help monitoring the effect of disease-modifying drugs.

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