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Sci Rep. 2018 Jan 29;8(1):1828. doi: 10.1038/s41598-018-19938-2.

MRI of the cervical spinal cord predicts respiratory dysfunction in ALS.

Author information

1
Service de Neurologie, Université de Lille, CHU de Lille, INSERM UMRS_1171, LICEND COEN Center, Lille, France.
2
Département de Biostastistiques, Université de Lille, CHU de Lille, Lille, France.
3
Service de Neuroradiologie, Université de Lille, CHU de Lille, INSERM UMRS_1171, LICEND COEN Center Lille, Lille, France.
4
Service de Pharmacologie, Médicale Université de Lille, CHU de Lille, INSERM UMRS_1171, LICEND COEN Center Lille, Lille, France.
5
Plateau d'imagerie préclinique, Université de Lille, CHU de Lille, Lille, France.
6
Department of Radiology, Neuroradiology section, Free University of Brussels, CHU Saint-Pierre, Brussels, Belgium.
7
Service de Pneumologie, Université de Lille, CHU de Lille, Lille, France.
8
Laboratoire d'Imagerie Biomédicale, CNRS, INSERM, Sorbonne Universités, UPMC Univ Paris 06, Paris, France.
9
Département de Neurologie, Centre référent SLA, APHP, Hôpital Pitié-Salpêtrière, Paris, France.
10
Service de Neurologie, Université de Lille, CHU de Lille, INSERM UMRS_1171, LICEND COEN Center, Lille, France. david.devos@chru-lille.fr.
11
Service de Pharmacologie, Médicale Université de Lille, CHU de Lille, INSERM UMRS_1171, LICEND COEN Center Lille, Lille, France. david.devos@chru-lille.fr.

Abstract

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.

TRIAL REGISTRATION:

ClinicalTrials.gov NCT02360891.

PMID:
29379040
PMCID:
PMC5789036
DOI:
10.1038/s41598-018-19938-2
[Indexed for MEDLINE]
Free PMC Article

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