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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

2D atrophy profile along the cervical spinal cord in SMA patients.(A) Cross-sectional area profile in SMA patients and controls. (B) Cord atrophy rate in SMA patients. The color-coding indicates regions with significant atrophy (color scale = p-values of the between groups comparison).
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pone.0152439.g003: 2D atrophy profile along the cervical spinal cord in SMA patients.(A) Cross-sectional area profile in SMA patients and controls. (B) Cord atrophy rate in SMA patients. The color-coding indicates regions with significant atrophy (color scale = p-values of the between groups comparison).

Mentions: Fig 3A and 3B show CSA profile from SMA patients and controls and cord atrophy rate along the cervical spinal cord, respectively. CSA showed a significant atrophy gradient mainly located between C3 and C6 vertebral levels with a cord atrophy rate ranging from 5.4% to 23%. CSA difference between SMA patients and controls significantly increased from C2 to C3 vertebral level and remained stable along C4 vertebral level with a mean cord atrophy rate equal to 20%. After this plateau, CSA difference decreased slowly and remained significant to the upper part of C7 vertebral level and was no longer significant beyond.


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)

2D atrophy profile along the cervical spinal cord in SMA patients.(A) Cross-sectional area profile in SMA patients and controls. (B) Cord atrophy rate in SMA patients. The color-coding indicates regions with significant atrophy (color scale = p-values of the between groups comparison).
© Copyright Policy
Related In: Results  -  Collection

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

pone.0152439.g003: 2D atrophy profile along the cervical spinal cord in SMA patients.(A) Cross-sectional area profile in SMA patients and controls. (B) Cord atrophy rate in SMA patients. The color-coding indicates regions with significant atrophy (color scale = p-values of the between groups comparison).
Mentions: Fig 3A and 3B show CSA profile from SMA patients and controls and cord atrophy rate along the cervical spinal cord, respectively. CSA showed a significant atrophy gradient mainly located between C3 and C6 vertebral levels with a cord atrophy rate ranging from 5.4% to 23%. CSA difference between SMA patients and controls significantly increased from C2 to C3 vertebral level and remained stable along C4 vertebral level with a mean cord atrophy rate equal to 20%. After this plateau, CSA difference decreased slowly and remained significant to the upper part of C7 vertebral level and was no longer significant beyond.

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