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

C. Ribstein; D. Courteix; N. Rabiau; C. Bommelaer; Y. Bourdeau; B. Pereira; Catherine Sarret

doi:10.1055/s-0038-1666846

Neuropediatrics, Table of Contents

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

Authors

C. Ribstein

¹Centre 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

²Laboratoire des Adaptations Métaboliques à l'Exercice en Conditions Physiologiques et Pathologiques (AME2P), Université Clermont Auvergne, Clermont-Ferrand, France

³CRNH Auvergne, Clermont-Ferrand, France
N. Rabiau

¹Centre de compétence des neuropathies de cause rare et centre de compétence des pathologies neuro-musculaires, Service de Génétique Médicale, Hôpital Estaing, Clermont-Ferrand, France

⁴INSERM-CIC 1405, Unité CRECHE, CHU Clermont-Ferrand, Clermont-Ferrand, France
C. Bommelaer

¹Centre 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

¹Centre 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

⁵Unité de Biostatistiques (DRCI), CHU Clermont-Ferrand, Clermont-Ferrand, France
Catherine Sarret

¹Centre de compétence des neuropathies de cause rare et centre de compétence des pathologies neuro-musculaires, Service de Génétique Médicale, Hôpital Estaing, Clermont-Ferrand, France

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

Full Text

References

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