Neuromuscular diseases and bone
- 1Multidisciplinary Department of Medical-Surgical and Dental Specialties, University of Campania Luigi Vanvitelli, Italy
Neuromuscular diseases (NMDs) are inherited or acquired conditions affecting skeletal muscles, motor nerves or neuromuscular junctions. Most of them are characterized by a progressive damage of muscle fibers with reduced muscle strength, disability and poor health-related quality of life of affected patients. In this scenario, skeletal health is usually compromised as a consequence of modified bone-muscle cross-talk including biomechanical and bio-humoral issues, resulting in increased risk of bone fragility and fractures. In addition NMDs patients frequently face nutritional issues, including malnutrition due to feeding disorders and swallowing problems that might affect bone health. Moreover, in these patients low levels of physical activity or immobility are common and might lead to overweight or obesity that can also interfere with bone strength features. Also vitamin D deficiency could play a critical role both in the pathogenesis and in the clinical scenario of many NMDs suggesting that its correction could be useful in maintaining or enhancing bone health, especially in the early phases of NMDs. Last but not least, specific disease-modifying drugs, available for some NMDs, are frequently burdened with adverse effects on bone tissue. For example, glucocorticoid therapy, standard of care for many muscular dystrophies, prolongs long-term survival in treated patients; nevertheless, high dose and/or chronic use of these drugs are common cause of secondary osteoporosis.
This review addresses the current state of knowledge about the factors that play a role in determining bone alterations reported in NMDs, how these factors can modify the biological pathways underlying bone health, and which are the available interventions to manage bone involvement in patients affected by NMDs.
Considering the complexity of care of these patients, an interdisciplinary and multimodal management strategy based on both pharmacological and non-pharmacological interventions is recommended, particularly targeting musculoskeletal issues that are closely related to functional independence as well as social implications.
Keywords: Neuromuscular Diseases, Osteoporosis, fractures, physical activity, Vitamin D, Glucocorticoids, Rehabilitation
Received: 11 Sep 2019;
Accepted: 30 Oct 2019.
Copyright: © 2019 Iolascon, Paoletta, Liguori, Curci and Moretti. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Prof. Giovanni Iolascon, Multidisciplinary Department of Medical-Surgical and Dental Specialties, University of Campania Luigi Vanvitelli, Naples, 80138, Campania, Italy, firstname.lastname@example.org