Axial muscle involvement in patients with limb girdle muscular dystrophy type R9

Introduction/Aims Limb girdle muscular dystrophy type R9 (LGMDR9) is characterized by progressive weakness of the shoulder and hip girdles. Involvement of proximal extremity muscles is well‐described whereas information about axial muscle involvement is lacking. It is important to recognize the invo...

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Bibliographic Details
Published in:Muscle & nerve 2022-04, Vol.65 (4), p.405-414
Main Authors: Revsbech, Karoline Lolk, Rudolf, Karen, Sheikh, Aisha Munawar, Khawajazada, Tahmina, Stricker Borch, Josefine, Dahlqvist, Julia Rebecka, Løkken, Nicoline, Witting, Nanna, Vissing, John
Format: Article
Language:English
Subjects:
axial myopathy
Dystrophy
Girdles
intramuscular fat
Leg
Legs
limb girdle muscular dystrophy
Magnetic resonance imaging
Muscles
Muscular dystrophy
paraspinal muscles
Patients
psoas major muscle
Thigh
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Summary:Introduction/Aims Limb girdle muscular dystrophy type R9 (LGMDR9) is characterized by progressive weakness of the shoulder and hip girdles. Involvement of proximal extremity muscles is well‐described whereas information about axial muscle involvement is lacking. It is important to recognize the involvement of axial muscles to understand functional challenges for the patients. The aim of this study was to investigate the involvement of axial and leg muscles in patients with LGMDR9. Methods This observational, cross‐sectional study investigated fat replacement of axial and leg muscles in 14 patients with LGMDR9 and 13 matched, healthy controls using quantitative MRI (Dixon technique). We investigated paraspinal muscles at three levels, psoas major at the lumbar level, and leg muscles in the thigh and calf. Trunk strength was assessed with stationary dynamometry and manual muscle tests. Results Patients with LGMDR9 had significantly increased fat replacement of all investigated axial muscles compared with healthy controls (P 
ISSN:0148-639X
1097-4598
DOI:10.1002/mus.27491