Neuromuscular diseases (NMDs) are inherited or acquired conditions affecting skeletalmuscles, motor nerves, or neuromuscular junctions. Most ofthem are characterizedby a progressive damage of muscle fibers with reduced muscle strength, disability,and poor health-related quality of life of affected patients. In this scenario, skeletalhealth is usually compromised as a consequence of modified bone–muscle cross-talk including biomechanical and bio-humoral issues, resulting in increased risk ofbone fragility and fractures. In addition, NMD patients frequently face nutritional issues,including malnutrition due to feeding disorders and swallowing problems that might affectbone health. Moreover, in these patients, low levels of physical activity or immobilityare common and might lead to overweight or obesity that can also interfere withbone strength features. Also, vitamin D deficiency could play a critical role both in thepathogenesis and in the clinical scenario of many NMDs, suggesting that its correctioncould be useful in maintaining or enhancing bone health, especially in the early phasesof NMDs. Last but not least, specific disease-modifying drugs, available for someNMDs, are frequently burdened with adverse effects on bone tissue. For example,glucocorticoid therapy, standard of care for many musculardystrophies, prolongs long-term survival in treated patients; nevertheless, high doseand/or chronic use of thesedrugs are a common cause of secondary osteoporosis. This review addresses thecurrent state of knowledge about the factors that play a rolein determining bonealterations reported in NMDs, how these factors can modify the biological pathwaysunderlying bone health, and which are the available interventions to manage boneinvolvement in patients affected by NMDs. Considering the complexity of care of thesepatients, an interdisciplinary and multimodal managementstrategy based on bothpharmacological and non-pharmacological interventions is recommended, particularlytargeting musculoskeletal issues that are closely relatedto functional independence aswell as social implications.
Neuromuscular Diseases and Bone
Iolascon G;Paoletta M;Liguori S;MORETTI A
2019
Abstract
Neuromuscular diseases (NMDs) are inherited or acquired conditions affecting skeletalmuscles, motor nerves, or neuromuscular junctions. Most ofthem are characterizedby a progressive damage of muscle fibers with reduced muscle strength, disability,and poor health-related quality of life of affected patients. In this scenario, skeletalhealth is usually compromised as a consequence of modified bone–muscle cross-talk including biomechanical and bio-humoral issues, resulting in increased risk ofbone fragility and fractures. In addition, NMD patients frequently face nutritional issues,including malnutrition due to feeding disorders and swallowing problems that might affectbone health. Moreover, in these patients, low levels of physical activity or immobilityare common and might lead to overweight or obesity that can also interfere withbone strength features. Also, vitamin D deficiency could play a critical role both in thepathogenesis and in the clinical scenario of many NMDs, suggesting that its correctioncould be useful in maintaining or enhancing bone health, especially in the early phasesof NMDs. Last but not least, specific disease-modifying drugs, available for someNMDs, are frequently burdened with adverse effects on bone tissue. For example,glucocorticoid therapy, standard of care for many musculardystrophies, prolongs long-term survival in treated patients; nevertheless, high doseand/or chronic use of thesedrugs are a common cause of secondary osteoporosis. This review addresses thecurrent state of knowledge about the factors that play a rolein determining bonealterations reported in NMDs, how these factors can modify the biological pathwaysunderlying bone health, and which are the available interventions to manage boneinvolvement in patients affected by NMDs. Considering the complexity of care of thesepatients, an interdisciplinary and multimodal managementstrategy based on bothpharmacological and non-pharmacological interventions is recommended, particularlytargeting musculoskeletal issues that are closely relatedto functional independence aswell as social implications.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
