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The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study

G. Querin, M. M. El Mendili, T. Lenglet, A. Behin, T. Stojkovic, F. Salachas, D. Devos, N. Le Forestier, M. Del Mar Amador, R. Debs, L. Lacomblez, V. Meininger, G. Bruneteau, Julien Cohen-Adad, S. Lehericy, P. Laforet, S. Blancho, H. Benali, M. Catala, M. Li, V. Marchand-Pauvert, J. Y. Hogrel, P. Bede, P. F. Pradat

Article (2019)

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Abstract

Spinal muscular atrophy (SMA) type III and IV are autosomal recessive, slowly progressive lower motor neuron syndromes. Nevertheless, wider cerebral involvement has been consistently reported in mouse models. The objective of this study is the characterisation of spinal and cerebral pathology in adult forms of SMA using multimodal quantitative imaging. METHODS: Twenty-five type III and IV adult SMA patients and 25 age-matched healthy controls were enrolled in a spinal cord and brain imaging study. Structural measures of grey and white matter involvement and diffusion parameters of white matter integrity were evaluated at each cervical spinal level. Whole-brain and region-of-interest analyses were also conducted in the brain to explore cortical thickness, grey matter density and tract-based white matter alterations. RESULTS: In the spinal cord, considerable grey matter atrophy was detected between C2-C6 vertebral levels. In the brain, increased grey matter density was detected in motor and extra-motor regions of SMA patients. No white matter pathology was identified neither at brain and spinal level. CONCLUSIONS: Adult forms of SMA are associated with selective grey matter degeneration in the spinal cord with preserved white matter integrity. The observed increased grey matter density in the motor cortex may represent adaptive reorganisation.

Uncontrolled Keywords

Adolescent; Adult; Aged; Brain/diagnostic imaging/*pathology; Diffusion Tensor Imaging; Female; Gray Matter/diagnostic imaging/pathology; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Muscular Atrophy, Spinal/diagnostic imaging/*pathology; Spinal Cord/diagnostic imaging/*pathology; Spinal Muscular Atrophies of Childhood/diagnostic imaging/*pathology; White Matter/diagnostic imaging/pathology; Young Adult; *Grey matter and white matter degeneration; *Multimodal MRI; *sma; *Spinal cord MRI; *Spinal muscular atrophy

Department: Institut de génie biomédical
Research Center: NeuroPoly - Laboratoire de Recherche en Neuroimagerie
Funders: Association Française contre les Myopathies (AFM), Institut pour la Recherche sur la Moelle épinière et l'Encéphale (IRME), Investissements d'avenir, Health Research Board, Irish Institute of Clinical Neuroscience IICN, Novartis Ireland Research Grant, Iris O'Brien Foundation Ireland
Grant number: ANR-10-IAIHU-06, HRB EIA-2017-019
PolyPublie URL: https://publications.polymtl.ca/5099/
Journal Title: NeuroImage: Clinical (vol. 21)
Publisher: Elsevier
DOI: 10.1016/j.nicl.2018.101618
Official URL: https://doi.org/10.1016/j.nicl.2018.101618
Date Deposited: 26 Sep 2022 11:39
Last Modified: 22 Nov 2022 00:55
Cite in APA 7: Querin, G., El Mendili, M. M., Lenglet, T., Behin, A., Stojkovic, T., Salachas, F., Devos, D., Le Forestier, N., Del Mar Amador, M., Debs, R., Lacomblez, L., Meininger, V., Bruneteau, G., Cohen-Adad, J., Lehericy, S., Laforet, P., Blancho, S., Benali, H., Catala, M., ... Pradat, P. F. (2019). The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study. NeuroImage: Clinical, 21, 101618. https://doi.org/10.1016/j.nicl.2018.101618

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