Title |
Cervical Spinal Cord Atrophy Profile in Adult SMN1-Linked SMA
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Published in |
PLOS ONE, April 2016
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DOI | 10.1371/journal.pone.0152439 |
Pubmed ID | |
Authors |
Mohamed-Mounir El Mendili, Timothée Lenglet, Tanya Stojkovic, Anthony Behin, Raquel Guimarães-Costa, François Salachas, Vincent Meininger, Gaelle Bruneteau, Nadine Le Forestier, Pascal Laforêt, Stéphane Lehéricy, Habib Benali, Pierre-François Pradat |
Abstract |
The mechanisms underlying the topography of motor deficits in spinal muscular atrophy (SMA) remain unknown. We investigated the profile of spinal cord atrophy (SCA) in SMN1-linked SMA, and its correlation with the topography of muscle weakness. Eighteen SMN1-linked SMA patients type III/V and 18 age/gender-matched healthy volunteers were included. Patients were scored on manual muscle testing and functional scales. Spinal cord was imaged using 3T MRI system. Radial distance (RD) and cord cross-sectional area (CSA) measurements in SMA patients were compared to those in controls and correlated with strength and disability scores. CSA measurements revealed a significant cord atrophy gradient mainly located between C3 and C6 vertebral levels with a SCA rate ranging from 5.4% to 23% in SMA patients compared to controls. RD was significantly lower in SMA patients compared to controls in the anterior-posterior direction with a maximum along C4 and C5 vertebral levels (p-values < 10-5). There were no correlations between atrophy measurements, strength and disability scores. Spinal cord atrophy in adult SMN1-linked SMA predominates in the segments innervating the proximal muscles. Additional factors such as neuromuscular junction or intrinsic skeletal muscle defects may play a role in more complex mechanisms underlying weakness in these patients. |
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United States | 1 | 100% |
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Members of the public | 1 | 100% |
Mendeley readers
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Unknown | 52 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Ph. D. Student | 15 | 29% |
Researcher | 5 | 10% |
Student > Bachelor | 4 | 8% |
Other | 4 | 8% |
Student > Master | 4 | 8% |
Other | 6 | 12% |
Unknown | 14 | 27% |
Readers by discipline | Count | As % |
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Medicine and Dentistry | 15 | 29% |
Neuroscience | 4 | 8% |
Engineering | 4 | 8% |
Chemistry | 2 | 4% |
Computer Science | 1 | 2% |
Other | 6 | 12% |
Unknown | 20 | 38% |