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Neuropediatrics 2018; 49(06): 397-400
DOI: 10.1055/s-0038-1666846
Short Communication
Georg Thieme Verlag KG Stuttgart · New York

Secondary Bone Defect in Neuromuscular Diseases in Childhood: A Longitudinal “Muscle-Bone Unit” Analysis

C. Ribstein
1   Centre de compétence des neuropathies de cause rare et centre de compétence des pathologies neuro-musculaires, Service de Génétique Médicale, Hôpital Estaing, Clermont-Ferrand, France
,
D. Courteix
2   Laboratoire des Adaptations Métaboliques à l'Exercice en Conditions Physiologiques et Pathologiques (AME2P), Université Clermont Auvergne, Clermont-Ferrand, France
3   CRNH Auvergne, Clermont-Ferrand, France
,
N. Rabiau
1   Centre de compétence des neuropathies de cause rare et centre de compétence des pathologies neuro-musculaires, Service de Génétique Médicale, Hôpital Estaing, Clermont-Ferrand, France
4   INSERM-CIC 1405, Unité CRECHE, CHU Clermont-Ferrand, Clermont-Ferrand, France
,
C. Bommelaer
1   Centre de compétence des neuropathies de cause rare et centre de compétence des pathologies neuro-musculaires, Service de Génétique Médicale, Hôpital Estaing, Clermont-Ferrand, France
,
Y. Bourdeau
1   Centre de compétence des neuropathies de cause rare et centre de compétence des pathologies neuro-musculaires, Service de Génétique Médicale, Hôpital Estaing, Clermont-Ferrand, France
,
B. Pereira
5   Unité de Biostatistiques (DRCI), CHU Clermont-Ferrand, Clermont-Ferrand, France
,
Catherine Sarret
1   Centre de compétence des neuropathies de cause rare et centre de compétence des pathologies neuro-musculaires, Service de Génétique Médicale, Hôpital Estaing, Clermont-Ferrand, France
› Author Affiliations
Further Information

Publication History

02 April 2018

30 May 2018

Publication Date:
06 July 2018 (online)

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Abstract

To evaluate the potential bone defect in neuromuscular diseases, we conducted a longitudinal study including three groups of patients: 14 Duchenne muscular dystrophies (DMD) and 2 limb-girdle muscular dystrophies (LGMD); 3 Becker muscular dystrophies (BeMD) and 7 spinal muscular atrophies (SMA). Yearly osteodensitometries assessed body composition and bone mineral density (BMD) associated with bone markers and leptin. Along the 7-year study, 107 osteodensitometries showed that bone status evolved to osteopenia in most patients except BeMD. When analyzing the crude values, BMD improved with age in BeMD and SMA but not in DMD/LGMD. The correlation using the Z-scores displayed a decrease in BMD with age in DMD/LGMD for all regions, in SMA at total body less head, whereas BMD increased in BeMD at lumbar spine. As observed in healthy persons, muscular mass and bone tissue were significantly correlated. Glucocorticoids were deleterious on trabecular and cortical bone. Leptin was high in most patients and correlated to fat mass and bone parameters. This study confirms a secondary bone defect in neuromuscular diseases, further confirming the functional relationship between bone and muscle and arguing for regular bone follow-up in patients to prevent fracture risk. Adipose tissue seems to interfere with bone remodeling in neuromuscular diseases.

Keywords

functional muscle-bone unit - neuromuscular diseases - bone mineral density - osteopenia - glucocorticoid - leptin

Financial Support

None.


 

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