Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 Jun 1;17(2):1-7.

Muscle abnormalities in osteogenesis imperfecta

L N Veilleux  1 , P Trejo, F Rauch
Affiliations
Review

Muscle abnormalities in osteogenesis imperfecta

L N Veilleux et al. J Musculoskelet Neuronal Interact. .

Abstract

Osteogenesis imperfecta (OI) is mainly characterized by bone fragility but muscle abnormalities have been reported both in OI mouse models and in children with OI. Muscle mass is decreased in OI, even when short stature is taken into account. Dynamic muscle tests aiming at maximal eccentric force production reveal functional deficits that can not be explained by low muscle mass alone. However, it appears that diaphyseal bone mass is normally adapted to muscle force. At present the determinants of muscle mass and function in OI have not been clearly defined. Physiotherapy interventions and bisphosphonate treatment appear to have some effect on muscle function in OI. Interventions targeting muscle mass have shown encouraging results in OI animal models and are an interesting area for further research.

PubMed Disclaimer

Conflict of interest statement

The authors have no conflict of interest.

Figures

Figure 1
Figure 1
Anteroposterior radiographs of lower extremities. (A) 11-year old girl with OI type I who has not received bisphosphonate treatment and has not had intramedullary rodding surgery. There is a mild deformity of the left femur following a fracture at the age of 12 months. (B) 10-year old girl with OI type IV. Intravenous bisphosphonates had been given since the age of 4 years and both femurs have intramedullary nails. Coxa vara corrective surgery had been performed on the the right femoral neck. (C) 10-year old girl with OI type III. She had received intravenous bisphosphonate treatment since the age of 2 months. Both femurs and tibias have undergone rodding surgery.
Figure 2
Figure 2
Peripheral quantitative computed tomography scan images at the 65% forearm site in a healthy control and in boys with OI. Cortical bone is shown in white, muscle in light grey and fat in dark grey. Bone CSA represents the cross-sectional area of the radius, including the marrow cavity. Muscle CSA is the cross-sectional area of the forearm muscle. FA length corresponds to the forearm length, as measured from the tip of the ulnar styloid process to the tip of the olecranon.
See this image and copyright information in PMC

References

    1. Berendsen AD, Olsen BR. Bone development. Bone. 2015;80:14–8. - PMC - PubMed
    1. Goodman CA, Hornberger TA, Robling AG. Bone and skeletal muscle: Key players in mechanotransduction and potential overlapping mechanisms. Bone. 2015;80:24–36. - PMC - PubMed
    1. Schonau E, Werhahn E, Schiedermaier U, Mokow E, Schiessl H, Scheidhauer K, et al. Influence of muscle strength on bone strength during childhood and adolescence. Horm Res. 1996;45(Suppl 1):63–6. - PubMed
    1. Wong BL, Rybalsky I, Shellenbarger KC, Tian C, McMahon MA, Rutter MM, et al. Long-term outcome of interdisciplinary management of patients with Duchenne muscular dystrophy receiving daily glucocorticoid treatment. J Pediatr. 2017;182:296–303e1. - PubMed
    1. Ma J, McMillan HJ, Karaguzel G, Goodin C, Wasson J, Matzinger MA, et al. The time to and determinants of first fractures in boys with Duchenne muscular dystrophy. Osteoporos Int. 2017;28:597–608. - PubMed

MeSH terms