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September 23, 2014; 83 (13) Article

Severe paraspinal muscle involvement in facioscapulohumeral muscular dystrophy

Julia R. Dahlqvist, Christoffer R. Vissing, Carsten Thomsen, John Vissing
First published August 20, 2014, DOI: https://doi.org/10.1212/WNL.0000000000000828
Julia R. Dahlqvist
From the Neuromuscular Research Unit, Department of Neurology (J.R.D., C.R.V., J.V.) and Department of Diagnostic Radiology (C.T.), Rigshospitalet, University of Copenhagen, Denmark.
MD
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Christoffer R. Vissing
From the Neuromuscular Research Unit, Department of Neurology (J.R.D., C.R.V., J.V.) and Department of Diagnostic Radiology (C.T.), Rigshospitalet, University of Copenhagen, Denmark.
BSc
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Carsten Thomsen
From the Neuromuscular Research Unit, Department of Neurology (J.R.D., C.R.V., J.V.) and Department of Diagnostic Radiology (C.T.), Rigshospitalet, University of Copenhagen, Denmark.
MD, PhD
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John Vissing
From the Neuromuscular Research Unit, Department of Neurology (J.R.D., C.R.V., J.V.) and Department of Diagnostic Radiology (C.T.), Rigshospitalet, University of Copenhagen, Denmark.
MD, PhD
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Citation
Severe paraspinal muscle involvement in facioscapulohumeral muscular dystrophy
Julia R. Dahlqvist, Christoffer R. Vissing, Carsten Thomsen, John Vissing
Neurology Sep 2014, 83 (13) 1178-1183; DOI: 10.1212/WNL.0000000000000828

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Abstract

Objective: In this study, involvement of paraspinal muscles in 50 patients with facioscapulohumeral dystrophy (FSHD) was evaluated using MRI.

Methods: The Dixon MRI technique was used in this observational study to quantify muscle fat content of paraspinal and leg muscles. Muscle strength in the neck, back, and legs was assessed with a handheld dynamometer. All subjects completed the Low Back Pain Rating Scale questionnaire. MRI findings were compared with 31 age-matched controls and correlated to muscle strength, back pain, and MRI findings in lower extremities.

Results: The fat fraction in muscles was significantly higher in patients with FSHD than in controls: paraspinal fat fraction was 38% in patients vs 20% in controls, thigh fat fraction was 36% vs 11%, and calf fat fraction was 37% vs 11%. Increased paraspinal fat fraction correlated with D4Z4 repeat size, FSHD severity score, fat fraction of the thigh, and muscle strength in the back. The prevalence of back pain was 3 times higher in patients with FSHD vs controls, but back pain did not correlate with the paraspinal fat fraction.

Conclusions: This study shows a prominent involvement of paraspinal muscles in patients with FSHD, which should be considered in the management of this condition.

GLOSSARY

DMD=
Duchenne muscular dystrophy;
FSHD=
facioscapulohumeral dystrophy;
LBPRS=
Low Back Pain Rating Scale;
TE=
echo time

Footnotes

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Supplemental data at Neurology.org

  • Received March 24, 2014.
  • Accepted in final form June 30, 2014.
  • © 2014 American Academy of Neurology
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