MRI of the cervical spinal cord predicts respiratory dysfunction in ALS

G. Grolez, M. Kyheng, R. Lopes, C. Moreau, K. Timmerman, F. Auger, G. Kuchcinski, A. Duhamel, P. Jissendi-Tchofo, P. Besson, C. Laloux, M. Petrault, J. C. Devedjian, Thierry Pérez, Pierre François Pradat, L. Defebvre, R. Bordet, V. Danel-Brunaud, D. Devos*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

22 Scopus citations


For patients with amyotrophic lateral sclerosis (ALS), the primary therapeutic goal is to minimize morbidity. Non-invasive ventilation improves survival. We aim to assess whether Magnetic Resonance Imaging (MRI) of the cervical spinal cord predicts the progression of respiratory disorders in ALS. Brain and spinal MRI was repeatedly performed in the SOD1G86R mouse model, in 40 patients and in healthy controls. Atrophy, iron overload, white matter diffusivity and neuronal loss were assessed. In Superoxide Dismutase-1 (SOD1) mice, iron accumulation appeared in the cervical spinal cord at symptom onset but disappeared with disease progression (after the onset of atrophy). In ALS patients, the volumes of the motor cortex and the medulla oblongata were already abnormally low at the time of diagnosis. Baseline diffusivity in the internal capsule was predictive of functional handicap. The decrease in cervical spinal cord volume from diagnosis to 3 months was predictive of the change in slow vital capacity at 12 months. MRI revealed marked abnormalities at the time of ALS diagnosis. Early atrophy of the cervical spinal cord may predict the progression of respiratory disorders, and so may be of value in patient care and as a primary endpoint in pilot neuroprotection studies.

Original languageEnglish (US)
Article number1828
JournalScientific reports
Issue number1
StatePublished - Dec 1 2018
Externally publishedYes

ASJC Scopus subject areas

  • General


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