Bone health and associated metabolic complications in neuromuscular diseases

Abstract

This article reviews the recent literature regarding bone health as it relates to the patient living with neuromuscular disease (NMD). Studies defining the scope of bone-related disease in NMD are scant. The available evidence is discussed, focusing on abnormal calcium metabolism, increased fracture risk, and the prevalence of both scoliosis and hypovitaminosis D in Duchenne muscular dystrophy, amyotrophic lateral sclerosis, and spinal muscular atrophy. Future directions are discussed, including the urgent need for studies both to determine the nature and extent of poor bone health, and to evaluate the therapeutic effect of available osteoporosis treatments in patients with NMD.

Figure 1

9-year-old boy with DMD treated with chronic glucocorticoids, who has scoliosis and a 3week history of intermittent lower back pain. MRI of his lumbar spine reveals multilevel vertebral body height loss with increased prominence of the disc spaces with respect to the vertebral bodies. At the superior endplate of L5, and L1, as well as diffusely in the L2 vertebral body there is increased T2 signal suggesting marrow edema and more recent fracture.

Figure 2

A radiograph of a 6-year-old with SMAII and history of scoliosis, revealing a 61-degree dextroscoliotic curvature of the thoracolumbar spine.