Key Principles and Disease-Specific Considerations to Guide Management of Bone Health and Osteoporosis Among Individuals With Neuromuscular Disorders: The Path Forward

Given the intimate link between muscle function and the skeleton's ability to adapt to mechanical loads, it is no surprise that individuals with neuromuscular disorders (NMDs) are at risk for low-trauma (i.e., osteoporotic) fragility fractures. In this review, we highlight the interdependent relationship between muscle and bone strength and the need to assess individuals with NMD who demonstrate muscle weakness that interferes with weight bearing and typical activities of daily living. This review also emphasizes the importance of the clinical context when evaluating the risk of bone fragility, as well as the potential to reclaim bone strength in the absence of bone-targeted therapy. Indeed, longitudinal skeletal phenotyping is key to understanding the individual's bone health trajectory and the need for progressive intensification (or de-escalation) of osteoporosis prevention and treatment. Overall, osteoporosis management has moved away from a bone mineral density (BMD)-centric approach to a fracture-focused approach, with vertebral fractures a clear indicator for bone-strengthening therapy (recognizing that they are frequently asymptomatic, necessitating periodic spine imaging for their timely identification). BMD assessments help guide the frequency of spine imaging and response to bone-targeted therapy and are best undertaken at multiple skeletal sites given the potential for “regional osteoporosis.” Multidisciplinary osteoporosis prevention and treatment, including an expert in skeletal health, is the cornerstone of effective osteoporosis management. Looking forward, the field is shifting from secondary osteoporosis prevention to a more proactive, anticipatory approach in those with persistent risk factors, one that involves initiation of bone-targeted therapy prior to first-ever fractures.