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.

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 http://study.com/academy/lesson/common-disorders-of-the- 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.

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.

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 https://www.spine-health.com/video/kyphosis-video-what- kyphosis https://goo.gl/images/gCwG3s 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.

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 https://www.youtube.com/watch?v=RR6GOatrPnA 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.

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 https://www.youtube.com/watch?v=ZrPnmgs4rHM 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.

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.

Disorders of the Musculoskeletal System Joint disorders: arthrogryposis https://www.youtube.com/watch?v=ks47VzS4gyY Congenital joint contractures prolonged fetal immobility Skeletal dysplasias: achondroplasia https://www.youtube.com/watch?v=EawZCTdD_lQ 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.

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.

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.

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.