Related Experiment Video

Updated: Aug 19, 2026

Behavioral and Locomotor Measurements Using an Open Field Activity Monitoring System for Skeletal Muscle Diseases
06:52

Behavioral and Locomotor Measurements Using an Open Field Activity Monitoring System for Skeletal Muscle Diseases

Published on: September 29, 2014

Skeletal changes in children with neuromuscular disorders

J D Hsu

    Progress in Clinical and Biological Research
    |January 1, 1982
    PubMed

    Insights

    Skeletal issues like thin bones, osteoporosis, and fractures are common in Duchenne muscular dystrophy and spinal muscular atrophy. Early recognition and proper handling are crucial for managing these bone and joint complications.

    Area of Science:

    • Orthopedics
    • Neuromuscular Disorders
    • Pediatric Skeletal Health

    Background:

    • Duchenne muscular dystrophy (DMD) and spinal muscular atrophy (SMA) are genetic neuromuscular disorders.
    • These conditions significantly impact muscle function, leading to skeletal complications.
    • Understanding these skeletal manifestations is vital for patient management.

    Purpose of the Study:

    • To document and analyze the skeletal findings in patients with DMD and SMA.
    • To highlight the characteristic bone and joint abnormalities associated with these diseases.
    • To inform clinical practice regarding the prevention and management of skeletal issues.

    Main Methods:

    • Retrospective analysis of skeletal findings.
    • Inclusion of 45 boys with Duchenne muscular dystrophy (ages 1-21).
    • Inclusion of 81 patients with spinal muscular atrophy (ages 1-35).

    Main Results:

    • Long bones exhibit thin cortices and osteoporosis in both patient groups.
    • Joints display distortions secondary to contractures.
    • Fractures occur with minimal trauma, affecting bones and joints.

    Conclusions:

    • Skeletal fragility, including osteoporosis and fractures, is a significant concern in DMD and SMA.
    • Proper positioning of extremities and spine is essential for treatment.
    • Careful handling of patients is critical for fracture prevention.

    More Related Videos

    Quantitative Magnetic Resonance Imaging of Skeletal Muscle Disease09:30

    Quantitative Magnetic Resonance Imaging of Skeletal Muscle Disease

    Published on: December 18, 2016

    Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy09:18

    Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy

    Published on: January 12, 2019

    Related Concept Videos

    The Functions of the Skeletal System01:22

    The Functions of the Skeletal System

    The most apparent functions of the skeletal system are support, protection, and movement. However, bone tissue also performs several other critical metabolic functions. For one, the bone matrix acts as a reservoir for a number of minerals important to the functioning of the body, especially calcium and phosphorus. These minerals, present in the bone tissue, can be released back into the bloodstream when required. Calcium ions, for example, are essential for muscle contractions and controlling...
    Bone Disorders01:29

    Bone Disorders

    Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
    Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
    Changes in the Appendicular Skeleton with Age01:09

    Changes in the Appendicular Skeleton with Age

    The upper and lower limb initially develops as a small bulge called a limb bud, which appears on the lateral side of the early embryo. The upper limb bud appears near the end of the fourth week of development, with the lower limb bud appearing shortly after.
    Initially, the limb buds consist of a core of mesenchyme covered by a layer of ectoderm. The ectoderm at the end of the limb bud thickens to form a narrow crest called the apical ectodermal ridge. This ridge stimulates the underlying...
    Disorders of the Skeletal Muscle01:28

    Disorders of the Skeletal Muscle

    The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
    Musculoskeletal disorders
    Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
    Alterations in Muscle Tone ll01:12

    Alterations in Muscle Tone ll

    Alterations in muscle tone are common manifestations of neurological disorders and reflect dysfunction within different nervous system regions. Spasticity, paratonia, and dystonia represent distinct forms of hypertonia, each with unique mechanisms, clinical features, and diagnostic importance.CharacteristicsSpasticity happens from upper motor neuron lesions and is characterized by velocity-dependent resistance to passive movement. Clinical features include:Exaggerated deep tendon reflexesClonus...
    Alterations in Muscle Tone lll01:11

    Alterations in Muscle Tone lll

    Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...