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Cervical Spinal Cord Atrophy Profile in Adult SMN1-Linked SMA.

El Mendili MM, Lenglet T, Stojkovic T, Behin A, Guimarães-Costa R, Salachas F, Meininger V, Bruneteau G, Le Forestier N, Laforêt P, Lehéricy S, Benali H, Pradat PF - PLoS ONE (2016)

Bottom Line: Patients were scored on manual muscle testing and functional scales.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.

View Article: PubMed Central - PubMed

Affiliation: Sorbonne Universités, UPMC Univ Paris 06, CNRS, INSERM, Laboratoire d'Imagerie Biomédicale, F-75013, Paris, France.

ABSTRACT

Purpose: 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.

Materials and methods: 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.

Results: 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.

Conclusions: 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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Related in: MedlinePlus

3D atrophy profile along the cervical spinal cord in SMA patients.The color-coding indicates regions with significant atrophy (color scale = p-values of the between groups comparison). A, anterior; I, inferior; L, left, P, posterior, R, right, S, superior.
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pone.0152439.g002: 3D atrophy profile along the cervical spinal cord in SMA patients.The color-coding indicates regions with significant atrophy (color scale = p-values of the between groups comparison). A, anterior; I, inferior; L, left, P, posterior, R, right, S, superior.

Mentions: Fig 2 shows the 3D p-values profile resulting from RD comparisons between SMA patients and controls. RD was significantly lower in SMA patients compared to controls in the anterior-posterior direction and mostly located between C3 and C6 vertebral levels, corresponding to C4–C7 spinal segments, with a maximum along C4 and C5 vertebral levels, corresponding to C5–C6 spinal segments, (p-values < 10−5) [19].


Cervical Spinal Cord Atrophy Profile in Adult SMN1-Linked SMA.

El Mendili MM, Lenglet T, Stojkovic T, Behin A, Guimarães-Costa R, Salachas F, Meininger V, Bruneteau G, Le Forestier N, Laforêt P, Lehéricy S, Benali H, Pradat PF - PLoS ONE (2016)

3D atrophy profile along the cervical spinal cord in SMA patients.The color-coding indicates regions with significant atrophy (color scale = p-values of the between groups comparison). A, anterior; I, inferior; L, left, P, posterior, R, right, S, superior.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4835076&req=5

pone.0152439.g002: 3D atrophy profile along the cervical spinal cord in SMA patients.The color-coding indicates regions with significant atrophy (color scale = p-values of the between groups comparison). A, anterior; I, inferior; L, left, P, posterior, R, right, S, superior.
Mentions: Fig 2 shows the 3D p-values profile resulting from RD comparisons between SMA patients and controls. RD was significantly lower in SMA patients compared to controls in the anterior-posterior direction and mostly located between C3 and C6 vertebral levels, corresponding to C4–C7 spinal segments, with a maximum along C4 and C5 vertebral levels, corresponding to C5–C6 spinal segments, (p-values < 10−5) [19].

Bottom Line: Patients were scored on manual muscle testing and functional scales.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.

View Article: PubMed Central - PubMed

Affiliation: Sorbonne Universités, UPMC Univ Paris 06, CNRS, INSERM, Laboratoire d'Imagerie Biomédicale, F-75013, Paris, France.

ABSTRACT

Purpose: 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.

Materials and methods: 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.

Results: 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.

Conclusions: 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.

Show MeSH
Related in: MedlinePlus