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The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study
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 adu...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6413472/ https://www.ncbi.nlm.nih.gov/pubmed/30522974 http://dx.doi.org/10.1016/j.nicl.2018.101618 |
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author | Querin, Giorgia El Mendili, Mohamed-Mounir Lenglet, Timothée Behin, Anthony Stojkovic, Tanya Salachas, François Devos, David Le Forestier, Nadine del Mar Amador, Maria Debs, Rabab Lacomblez, Lucette Meninger, Vincent Bruneteau, Gaëlle Cohen-Adad, Julien Lehéricy, Stéphane Laforêt, Pascal Blancho, Sophie Benali, Habib Catala, Martin Li, Menghan Marchand-Pauvert, Véronique Hogrel, Jean-Yves Bede, Peter Pradat, Pierre-François |
author_facet | Querin, Giorgia El Mendili, Mohamed-Mounir Lenglet, Timothée Behin, Anthony Stojkovic, Tanya Salachas, François Devos, David Le Forestier, Nadine del Mar Amador, Maria Debs, Rabab Lacomblez, Lucette Meninger, Vincent Bruneteau, Gaëlle Cohen-Adad, Julien Lehéricy, Stéphane Laforêt, Pascal Blancho, Sophie Benali, Habib Catala, Martin Li, Menghan Marchand-Pauvert, Véronique Hogrel, Jean-Yves Bede, Peter Pradat, Pierre-François |
author_sort | Querin, Giorgia |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-6413472 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-64134722019-03-22 The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study Querin, Giorgia El Mendili, Mohamed-Mounir Lenglet, Timothée Behin, Anthony Stojkovic, Tanya Salachas, François Devos, David Le Forestier, Nadine del Mar Amador, Maria Debs, Rabab Lacomblez, Lucette Meninger, Vincent Bruneteau, Gaëlle Cohen-Adad, Julien Lehéricy, Stéphane Laforêt, Pascal Blancho, Sophie Benali, Habib Catala, Martin Li, Menghan Marchand-Pauvert, Véronique Hogrel, Jean-Yves Bede, Peter Pradat, Pierre-François Neuroimage Clin Article 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. Elsevier 2018-11-28 /pmc/articles/PMC6413472/ /pubmed/30522974 http://dx.doi.org/10.1016/j.nicl.2018.101618 Text en © 2018 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Article Querin, Giorgia El Mendili, Mohamed-Mounir Lenglet, Timothée Behin, Anthony Stojkovic, Tanya Salachas, François Devos, David Le Forestier, Nadine del Mar Amador, Maria Debs, Rabab Lacomblez, Lucette Meninger, Vincent Bruneteau, Gaëlle Cohen-Adad, Julien Lehéricy, Stéphane Laforêt, Pascal Blancho, Sophie Benali, Habib Catala, Martin Li, Menghan Marchand-Pauvert, Véronique Hogrel, Jean-Yves Bede, Peter Pradat, Pierre-François The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study |
title | The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study |
title_full | The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study |
title_fullStr | The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study |
title_full_unstemmed | The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study |
title_short | The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study |
title_sort | spinal and cerebral profile of adult spinal-muscular atrophy: a multimodal imaging study |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6413472/ https://www.ncbi.nlm.nih.gov/pubmed/30522974 http://dx.doi.org/10.1016/j.nicl.2018.101618 |
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