1. Chapter 13 Muscles, Bones, and Nerves
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

2. Components of the Neuromuscular and Musculoskeletal Systems
Neuromuscular system connects the brain and spinal cord to the musculoskeletal system.
Musculoskeletal system consists of the skeletal muscles, tendons, bones, joints, and ligaments
These two systems are responsible for our ability to sit, stand, and move; impairments of these systems are major causes of developmental disability
skeletal-system.html
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

3. Symptoms and Signs of Neuromuscular and Musculoskeletal Disorders
Respiratory and cardiac issues
Rare but may be early signs of disorder
Muscle bulk, tone, and strength
Pseudohypertrophy (abnormal muscle enlargement) with weakness may be a sign of Duchenne muscular dystrophy (DMD)
Many disorders are linked to disturbances in muscle tone: hypertonia (high tone and an overreactive response to a normal stimulus) or hypotonia (low tone with a lesser response to a stimulus)
Muscle weaknesses may be proximal or distal
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

4. Symptoms and Signs of Neuromuscular and Musculoskeletal Disorders (continued)
Sensation: sensory symptoms such as numbness, paresthesia, and pain
Scoliosis, kyphosis, and lordosis: spinal curvatures may be complications of a neuromuscular or musculoskeletal disorder kyphosis
Contractures and gait abnormalities: joint contractures can develop (fixed loss of joint motion), and gait abnormalities (toe-walking, limping, walking with a wide-based gait) should be evaluated promptly
Joint abnormalities: loss of hip abduction may indicate a hip dislocation
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

5. Disorders of the Neuromuscular System
Anterior horn cells: spinal muscular atrophy (SMA)
Characterized by degeneration of a-motor neurons in the brainstem and spinal cord, leading to progressive weakness
Most common inherited cause of death in infancy
Disorders of the peripheral nerves: Charcot-Marie-Tooth (CMT) disease
Peripheral neuropathies may be motor, sensory, or sensorimotor
Patients with CMT typically present with weakness and wasting of the foot and lower leg muscles
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

6. Disorders of the Neuromuscular System (continued)
Diseases of the neuromuscular junction: myasthenia gravis
Acquired autoimmune disorders
Treatment generally targets improving neuromuscular transmission and increasing muscle strength while suppressing the production of abnormal antibodies
Disorders of muscle: muscular dystrophies and myopathies
Muscular dystrophies characterized by progressive destruction of muscle tissue; congenital myopathies involve either abnormal accumulation of certain proteins or other developmental abnormalities in the muscle fibers
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

7. Disorders of the Neuromuscular System (continued)
Duchenne muscular dystrophy
Progressive skeletal muscle disorder caused by mutations in the X-linked DMD gene
Accelerated deterioration in strength and balance
Cardiac and respiratory complications present in later stages of the disease; one of these usually constitutes the immediate cause of death
Congenital myopathies
Usually present in infancy with marked hypotonia and weakness, feeding difficulties, respiratory insufficiency
Associated with cranial nerve involvement and early respiratory complications
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

8. Disorders of the Musculoskeletal System
Joint disorders: arthrogryposis
Congenital joint contractures
prolonged fetal immobility
Skeletal dysplasias: achondroplasia
Abnormalities in development, growth, and maintenance of the skeleton; man
y disorders result in disproportionate short stature
Connective tissue disorders: osteogenesis imperfecta (OI)
Mechanical failure of collagen: joint hypermobility
Children with OI have brittle bones
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

9. Principles for the Management of Neuromuscular and Musculoskeletal Disorders
Medical care
Autoimmune neuromuscular disorders typically respond to immunomodulatory therapies (plasmapheresis, intravenous immunoglobulin, and steroids)
Therapies for inherited neuromuscular disorders are limited, but some medications are effective
Rehabilitation management
Active, nonresistive exercises (such as swimming) and an active lifestyle; walks to help maintain strength and slow contracture formation; wheelchair games
Physical therapy, occupational therapy to maintain activities of daily living, assistive technology for school
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

10. Principles for the Management of Neuromuscular and Musculoskeletal Disorders (continued)
Contractures
Stretching exercises and nighttime splinting as soon as tightness of the heel cords is noticed
Stretching, bracing, surgical release of tight heel cords
Bone health
Pathologic fractures (result from bone fragility, as a secondary effect of a medication, or weakness from disuse)
Weight-bearing activities for maintaining bone density
Diets rich in calcium and vitamin D, with supplementation of vitamin D in some cases
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

11. Principles for the Management of Neuromuscular and Musculoskeletal Disorders (continued)
Educational services
Children with acute neuromuscular disorders may require prolonged hospital stays and homebound instruction during convalescence
Children with fluctuating disorders may need rest periods or a shortened school day
Steroids used to treat neuromuscular diseases can have the side effect of causing behavior problems (potentially impacting school performance and personal relationships) and weight gain
Chapter 13 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.