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CHAPTER 51
ALTERATIONS IN
MUSCULOSKELETAL FUNCTION:
TRAUMA, INFECTION, AND
DISEASE
BONE AND JOINT TRAUMA
• Bones protect internal organs, contribute to
mineral homeostasis, produce blood cells,
and provide muscle attachment sites that
facilitate body movement
• Bone is one of the body’s hardest structures,
and most dynamic and metabolically active
tissues
• It is vascular with a capacity for repair
TYPES OF BONE
• Cortical bone forms the cortex (outer shell) of
the bone; when tension stress exceeds the
tolerance of cortical bone, fracture can
occur on the convex side of the bend
• Cancellous bone is found in the interior of the
bone and has a spongy, or lattice-like
appearance; provides structural support but
does not tolerate compression stress
FRACTURE
• A fracture is a break in continuity of a bone,
an epiphyseal plate, or a cartilaginous joint
surface
• Trauma may also occur to adjacent tissue
FRACTURE (CONT.)
TYPES OF FRACTURE
• Different types of fracture depend on the
type of tension stress involved
• Fractures can be classified according to the
orientation of the break as transverse,
longitudinal, oblique, or spiral
• A comminuted fracture consists of more than
one fracture line and more than two bone
fragments
• A greenstick fracture is an incomplete break
EXTENT OF FRACTURE
• A nondisplaced fracture occurs when the
fragments remain in alignment and position,
whereas a displaced fracture is one in which
the ends of fracture fragments are separated
• Fractures are classified as open or compound
when the skin is penetrated and as closed or
simple when the skin is not broken
HEALING PROCESS
• Cortical bone fracture results in torn blood
vessels within the haversian system
• Clotting occurs at the fracture site
• Avascular bone is replaced by living bone
through resorption and bone deposition
• Callus formation leads to new bone formation
at the edges of the periosteum
• Remodeling occurs with replacement of
mature lamellar bone
HEALING PROCESS (CONT.)
COMPLICATIONS IN BONE
HEALING
• Delayed healing can include delayed union,
malunion, or nonunion
• Treatment includes bone grafting,
internal/external fixation, electrical bone
stimulation, or a mixture of these
• Soft tissue complications may include
compartment syndrome or neovascular injury
DISLOCATIONS AND
SUBLUXATIONS
• Complete separation of joint articulating
surfaces is termed dislocation
• Subluxation refers to partial separation
• A considerable amount of tissue damage
occurs with dislocation or subluxation
• Evaluation of fracture is necessary
SCOLIOSIS
• Lateral curvature of the spine resulting in an S-
or a C-shaped spinal column with vertebral
column
• Nonstructural scoliosis resolves when the
patient bends to the affected side; structural
scoliosis fails to correct itself on forced
bending against the curvature and has
vertebral rotation
• Treatment may include bracing, exercises, or
surgical interventions with spinal alignment,
fusion, and internal fixation
ALTERATIONS IN BONE MASS
AND STRUCTURE
OSTEOPOROSIS
• The most common metabolic disease that
occurs when the rate of bone resorption is
greater than that of bone formation
• Hormone deficiencies (estrogen, androgen),
poor calcium intake, and lack of use are
common factors in the rate of bone loss
• Treatment may include calcium and vitamin
D supplements, exercise, estrogen
replacement therapy, bisphosphonates, or
recombinant human parathyroid hormone
RICKETS AND OSTEOMALACIA
• Vitamin D deficiency is associated with rickets
and osteomalacia, disorders characterized
by soft, weak bones
• Osteomalacia due to an inadequate
concentration of vitamin D, calcium or
phosphorus, poor vitamin D metabolism,
and/or renal disease
• Treatment includes vitamin D, calcium, or
phosphorus supplementation
PAGET DISEASE
• May be genetic or due to a viral infection
that affects osteoclastic function leading to
aberrant bone remodeling
• Painful bone deformities or bone fractures
may develop
• During active stages treatment is focused on
preventing deformity and fracture with
calcitonin or bisphosphonates
INFECTIONS OF THE BONE
• Hematogenous osteomyelitis occurs when
the infectious agent is introduced by blood
from infection elsewhere in the body
• May occur due to burns, sinus disease,
trauma, malignant tumor necrosis,
periodontal infection or an infected pressure
ulcer
• Staphylococcus aureus most common
pathogen
INFECTIONS OF THE BONE
(CONT.)
• Direct infection can occur due to open
fracture, penetrating wounds, surgical
contamination, or insertion of prostheses,
metal plates, or screws
OSTEOMYELITIS
• Severe pyogenic infection of bone and local
tissue
• Organisms reach the bone through the
bloodstream, adjacent soft tissue or direct
introduction of the organism into the bone
• Treatment: 4-6 wk of antibiotic therapy;
abscess formation may require debridement;
chronic infection may occur
TUBERCULOSIS
• Infection is spread via lung or
lymphohematogenous drainage due to
Mycobacterium tuberculosis
• Most common site is the vertebral column
• Treatment includes long-term combination
antibiotic therapy for 9-12 months; may be
complicated by drug resistance; surgical
intervention may be needed for severe
deformities or neurologic deficits
BONE TUMORS
• Neoplasms can be benign or malignant
• Malignant neoplasms are referred to as
sarcomas
• Metastatic lesions spread to bone from
primary carcinomas
• Common sites are the vertebral bodies,
pelvis, proximal ends of the femur and
humerus, and ribs
OSTEOCHONDROMA
• Most common bone tumor
• Cartilage-forming benign tumor that is
hereditary and usually occurs in those >30
years of age
• Pressure on surrounding tissue can cause pain
• Usually located on the metaphyses of long
bones, such as the proximal end of the tibia
and distal part of the femur, the shoulder,
and pelvis
CHONDROMA
• Cartilage-forming tumor in bone that can be
located in the medullary cavity or in the
subperiosteal layers of bone
• Arises from remnants of epiphyseal cartilage
• Typically located in the small bones of the
hands and feet
• May erode cortex of bone and expand the
contour
• Usually occurs in those 30-40 years of age
OSTEOID OSTEOMA
• A painful but benign bone-forming tumor
• Pain is usually sharp or dull, worse at night,
and alleviated by aspirin or NSAIDs
• Often found in the cortex of the tibia and
femur
• Usually occurs in those 10-20 years of age
GIANT CELL TUMOR
• A benign but aggressive tumor with richly
vascularized tissue consisting of plump
spindle-shaped cells and numerous giant cells
• Can undergo transformation to sarcomas
• Occurs between 20 and 40 years of age
• Located in distal end of the femur, proximal
end of the tibia, distal part of the radius, and
proximal end of the humerus
OSTEOSARCOMA
• Extremely malignant bone-forming tumor and
most common primary malignant bone tumor
• Formation of bone or osteoid by tumor cells
• Occurs in children and young adults
• Located in distal end of the femur, proximal
end of the tibia, fibula, humerus and flat
bones of the pelvis, skull, scapula, ribs, or
spine
OSTEOSARCOMA (CONT.)
• Treated with conservative surgery and
chemotherapy; may require amputation
CHONDROSARCOMA
• Malignant cartilage-forming tumor that
develops slowly; tends to develop in the
pelvic and shoulder girdles and the proximal
ends of long bones
• Characterized by formation of cartilage by
tumor cells
• Occurs in those 30-60 years of age
EWING SARCOMA
• Third most common primary sarcoma; a
rapidly growing malignant round cell tumor
• Develops in the long bones of children
• Composed of densely packed small cells with
round nuclei, may metastasize to lungs; pain
is a dominant symptom
• Treatment includes radiotherapy and possible
adjunct surgical therapy
MULTIPLE MYELOMA
• Slowly growing bone marrow malignancy
with neoplastic proliferation of a single clone
of plasma cells
• Homogeneous immunoglobulin is present in
urine and serum
• Pain is predominant symptom
• Treatment often requires aggressive
combination chemotherapy or local
radiation
SOFT-TISSUE INJURIES
• Contractile tissue is composed of structures
involved in the contraction of muscle and
includes not only the muscle belly but also the
tendon and bony insertion
• Inert tissue possesses no ability to contract or
relax
LIGAMENT INJURIES
• Occur when loading exceeds the physiologic
range of motion
• Microfailure precedes total failure
• Classified by extent of tear: mild, moderate,
or severe
• Symptoms occur as pain with weight bearing
and acute swelling
• Treatment dependent on degree of tear;
surgical restoration of ligament continuity
may be required in severe injury
JOINT CAPSULE INJURIES
• Injury causes increase in vascularity and
development of fibrous tissue leads to a thick
capsule
• Injury or edema in the joint that causes
scarring in the lax section of the capsule
prevents full range of motion
• Altered flow of synovial fluid prevents fluid
diffusion into and out of cartilage and causes
compression and distention
JOINT CAPSULE
INJURIES TO FASCIAE AND
BURSAE
• Fasciae are connective tissues of the body
arranged in sheaths that envelop muscles
• Bursae are pockets of connective tissue lined
with synovium in locations between muscles
or between muscle or tendon and bone
INJURIES TO NERVES, NERVE
ROOTS, AND DURA MATER
• Trauma to any soft tissue may lead to
adhesive constriction of the nerve, nerve
root, or dura mater
• Irritation or entrapment of a nerve causes
pain that radiates along the structures
innervated by the nerve
• Symptoms include altered sensation, motor
weakness, and diminished reflexes
INJURIES TO NERVES, NERVE
ROOTS, AND DURA MATER
(CONT.)
INJURY TO TENDONS
• Range from mild to complete tear
• Tendinitis may develop from infection, direct
injury or injury from repetitive motion
• Tendons are injured when the stress placed
on them is greater than the fibers can
tolerate
MUSCLE AND TENDON
STRAINS
• Categorized by the severity of injury
• Grade I: minute tear of connective tissue and
muscle fiber
• Grade II: tear of a large portion of the contractile
unit, with a segment still intact
• Grade III: total rupture or loss of continuity of the
contractile structure
BLUNT TRAUMA
• Can cause bleeding into the muscle belly;
may lead to an inability to contract the
muscle; may coagulate and calcify; results in
myositis ossificans
• Calcification prevents a normal and strong
contraction of the muscle involved
COMPARTMENT SYNDROME
• Results from trauma to soft tissue caused by
the unyielding structure of inert tissue
• Causes: decreased compartment size,
increased compartment content, or
externally applied pressure
• Edema causes increase in pressure in the
compartment; pressure reduces capillary
flow; muscle and nerves become ischemic
• Symptoms may include pain, paresthesia,
pale skin, and diminished pulse
EVALUATION OF
CONTRACTILE INJURIES
• Patterns of function deal with pain and
strength rather than excessive motion noted
during evaluation of ligamentous injury
• Strong and pain free
• Strong and painful
• Weak and painful
• Weak and pain free
SOFT-TISSUE HEALING AFTER
TRAUMA
• Disruption of circulatory and lymphatic
systems; hemorrhage, fluid loss, cell death
• Norepinephrine causes constriction of vessels;
serotonin and platelets prolong
vasoconstriction
• Inflammation; release of histamines causes
vasodilation and increased permeability of
venules
WOUND REPAIR
• Inflammatory response prepares injured tissue
• Granulation tissue develops in the wound
space
• Reepithelialization of the wound surface
occurs; formation of basement membrane
• Wound tensile strength is a result of deposition
of collagen
• Revascularization ensures survival of the new
tissue
WOUND REPAIR (CONT.)
• Wound closure or contraction is the final
phase of healing
DISEASES OF SKELETAL MUSCLE
• Skeletal muscle most abundant tissue in the
body; accounts for 40% of body weight
• Skeletal muscle performs dynamic and static
work
• Muscle atrophies in response to disuse and
immobilization, and hypertrophies in response
to increased stress
MUSCULAR DYSTROPHY
• A group of genetically determined
myopathies characterized by progressive
muscle weakness and degeneration
• Muscle tissue is replaced by fat and fibrous
connective tissue
• Classified by the pattern of inheritance, age
of onset, and distribution of muscular
weakness
DUCHENNE MUSCULAR
DYSTROPHY
• Most common and most severe form
• Inherited as X-linked trait, thus affects only
men
• Muscle cells are deficient in the protein
dystrophin; weakens the cell membrane and
allows extracellular fluid to leak into the cell
• Calf muscles enlarged, distal muscle
involvement
• Treatment includes corticosteroid therapy
and preservation of function
BECKER MUSCULAR
DYSTROPHY
• Milder form of inherited muscle degeneration
• Genetic mutation leads to production of a
reduced amount of an abnormal dystrophin
protein and slower muscular degeneration
• Calf hypertrophy prominent, often painful,
with progressive loss of strength and ability to
ambulate
FACIOSCAPULOHUMERAL
MUSCULAR DYSTROPHY
• Rare inherited autosomal dominant trait that
affects the muscles of the shoulder girdle and
face
• Facial muscles involved early with later
involvement of scapular and upper arm
musculature
• Progresses slowly with periods of arrest and
can ultimately involve more distal muscles
MYASTHENIA GRAVIS
• Chronic autoimmune disease; affects the
neuromuscular function of voluntary muscles
and characterized by profound muscle
weakness and fatigability
• Acetylcholine receptor antibodies produced
that destroy or block acetylcholine receptors
of the muscle end-plate of the neuromuscular
junction
MYASTHENIA GRAVIS (CONT.)
• Anticholinesterase inhibitors, corticosteroids,
intravenous immune globulin, plasmapheresis,
and immunosuppressive agents may be used
FIBROMYALGIA SYNDROME
• Cause remains unknown
• Characterized by widespread pain in all
quadrants of the body; additional symptoms
may include fatigue, insomnia, depression,
and concentration problems
• Treatment is multidimensional including
exercise, counseling, and drug therapy to
improve sleep, mood, and pain

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Pathophysiology Chapter 51

  • 1. CHAPTER 51 ALTERATIONS IN MUSCULOSKELETAL FUNCTION: TRAUMA, INFECTION, AND DISEASE
  • 2. BONE AND JOINT TRAUMA • Bones protect internal organs, contribute to mineral homeostasis, produce blood cells, and provide muscle attachment sites that facilitate body movement • Bone is one of the body’s hardest structures, and most dynamic and metabolically active tissues • It is vascular with a capacity for repair
  • 3. TYPES OF BONE • Cortical bone forms the cortex (outer shell) of the bone; when tension stress exceeds the tolerance of cortical bone, fracture can occur on the convex side of the bend • Cancellous bone is found in the interior of the bone and has a spongy, or lattice-like appearance; provides structural support but does not tolerate compression stress
  • 4. FRACTURE • A fracture is a break in continuity of a bone, an epiphyseal plate, or a cartilaginous joint surface • Trauma may also occur to adjacent tissue
  • 6. TYPES OF FRACTURE • Different types of fracture depend on the type of tension stress involved • Fractures can be classified according to the orientation of the break as transverse, longitudinal, oblique, or spiral • A comminuted fracture consists of more than one fracture line and more than two bone fragments • A greenstick fracture is an incomplete break
  • 7. EXTENT OF FRACTURE • A nondisplaced fracture occurs when the fragments remain in alignment and position, whereas a displaced fracture is one in which the ends of fracture fragments are separated • Fractures are classified as open or compound when the skin is penetrated and as closed or simple when the skin is not broken
  • 8. HEALING PROCESS • Cortical bone fracture results in torn blood vessels within the haversian system • Clotting occurs at the fracture site • Avascular bone is replaced by living bone through resorption and bone deposition • Callus formation leads to new bone formation at the edges of the periosteum • Remodeling occurs with replacement of mature lamellar bone
  • 10. COMPLICATIONS IN BONE HEALING • Delayed healing can include delayed union, malunion, or nonunion • Treatment includes bone grafting, internal/external fixation, electrical bone stimulation, or a mixture of these • Soft tissue complications may include compartment syndrome or neovascular injury
  • 11. DISLOCATIONS AND SUBLUXATIONS • Complete separation of joint articulating surfaces is termed dislocation • Subluxation refers to partial separation • A considerable amount of tissue damage occurs with dislocation or subluxation • Evaluation of fracture is necessary
  • 12. SCOLIOSIS • Lateral curvature of the spine resulting in an S- or a C-shaped spinal column with vertebral column • Nonstructural scoliosis resolves when the patient bends to the affected side; structural scoliosis fails to correct itself on forced bending against the curvature and has vertebral rotation • Treatment may include bracing, exercises, or surgical interventions with spinal alignment, fusion, and internal fixation
  • 13. ALTERATIONS IN BONE MASS AND STRUCTURE
  • 14. OSTEOPOROSIS • The most common metabolic disease that occurs when the rate of bone resorption is greater than that of bone formation • Hormone deficiencies (estrogen, androgen), poor calcium intake, and lack of use are common factors in the rate of bone loss • Treatment may include calcium and vitamin D supplements, exercise, estrogen replacement therapy, bisphosphonates, or recombinant human parathyroid hormone
  • 15. RICKETS AND OSTEOMALACIA • Vitamin D deficiency is associated with rickets and osteomalacia, disorders characterized by soft, weak bones • Osteomalacia due to an inadequate concentration of vitamin D, calcium or phosphorus, poor vitamin D metabolism, and/or renal disease • Treatment includes vitamin D, calcium, or phosphorus supplementation
  • 16. PAGET DISEASE • May be genetic or due to a viral infection that affects osteoclastic function leading to aberrant bone remodeling • Painful bone deformities or bone fractures may develop • During active stages treatment is focused on preventing deformity and fracture with calcitonin or bisphosphonates
  • 17. INFECTIONS OF THE BONE • Hematogenous osteomyelitis occurs when the infectious agent is introduced by blood from infection elsewhere in the body • May occur due to burns, sinus disease, trauma, malignant tumor necrosis, periodontal infection or an infected pressure ulcer • Staphylococcus aureus most common pathogen
  • 18. INFECTIONS OF THE BONE (CONT.) • Direct infection can occur due to open fracture, penetrating wounds, surgical contamination, or insertion of prostheses, metal plates, or screws
  • 19. OSTEOMYELITIS • Severe pyogenic infection of bone and local tissue • Organisms reach the bone through the bloodstream, adjacent soft tissue or direct introduction of the organism into the bone • Treatment: 4-6 wk of antibiotic therapy; abscess formation may require debridement; chronic infection may occur
  • 20. TUBERCULOSIS • Infection is spread via lung or lymphohematogenous drainage due to Mycobacterium tuberculosis • Most common site is the vertebral column • Treatment includes long-term combination antibiotic therapy for 9-12 months; may be complicated by drug resistance; surgical intervention may be needed for severe deformities or neurologic deficits
  • 21. BONE TUMORS • Neoplasms can be benign or malignant • Malignant neoplasms are referred to as sarcomas • Metastatic lesions spread to bone from primary carcinomas • Common sites are the vertebral bodies, pelvis, proximal ends of the femur and humerus, and ribs
  • 22. OSTEOCHONDROMA • Most common bone tumor • Cartilage-forming benign tumor that is hereditary and usually occurs in those >30 years of age • Pressure on surrounding tissue can cause pain • Usually located on the metaphyses of long bones, such as the proximal end of the tibia and distal part of the femur, the shoulder, and pelvis
  • 23. CHONDROMA • Cartilage-forming tumor in bone that can be located in the medullary cavity or in the subperiosteal layers of bone • Arises from remnants of epiphyseal cartilage • Typically located in the small bones of the hands and feet • May erode cortex of bone and expand the contour • Usually occurs in those 30-40 years of age
  • 24. OSTEOID OSTEOMA • A painful but benign bone-forming tumor • Pain is usually sharp or dull, worse at night, and alleviated by aspirin or NSAIDs • Often found in the cortex of the tibia and femur • Usually occurs in those 10-20 years of age
  • 25. GIANT CELL TUMOR • A benign but aggressive tumor with richly vascularized tissue consisting of plump spindle-shaped cells and numerous giant cells • Can undergo transformation to sarcomas • Occurs between 20 and 40 years of age • Located in distal end of the femur, proximal end of the tibia, distal part of the radius, and proximal end of the humerus
  • 26. OSTEOSARCOMA • Extremely malignant bone-forming tumor and most common primary malignant bone tumor • Formation of bone or osteoid by tumor cells • Occurs in children and young adults • Located in distal end of the femur, proximal end of the tibia, fibula, humerus and flat bones of the pelvis, skull, scapula, ribs, or spine
  • 27. OSTEOSARCOMA (CONT.) • Treated with conservative surgery and chemotherapy; may require amputation
  • 28. CHONDROSARCOMA • Malignant cartilage-forming tumor that develops slowly; tends to develop in the pelvic and shoulder girdles and the proximal ends of long bones • Characterized by formation of cartilage by tumor cells • Occurs in those 30-60 years of age
  • 29. EWING SARCOMA • Third most common primary sarcoma; a rapidly growing malignant round cell tumor • Develops in the long bones of children • Composed of densely packed small cells with round nuclei, may metastasize to lungs; pain is a dominant symptom • Treatment includes radiotherapy and possible adjunct surgical therapy
  • 30. MULTIPLE MYELOMA • Slowly growing bone marrow malignancy with neoplastic proliferation of a single clone of plasma cells • Homogeneous immunoglobulin is present in urine and serum • Pain is predominant symptom • Treatment often requires aggressive combination chemotherapy or local radiation
  • 31. SOFT-TISSUE INJURIES • Contractile tissue is composed of structures involved in the contraction of muscle and includes not only the muscle belly but also the tendon and bony insertion • Inert tissue possesses no ability to contract or relax
  • 32. LIGAMENT INJURIES • Occur when loading exceeds the physiologic range of motion • Microfailure precedes total failure • Classified by extent of tear: mild, moderate, or severe • Symptoms occur as pain with weight bearing and acute swelling • Treatment dependent on degree of tear; surgical restoration of ligament continuity may be required in severe injury
  • 33. JOINT CAPSULE INJURIES • Injury causes increase in vascularity and development of fibrous tissue leads to a thick capsule • Injury or edema in the joint that causes scarring in the lax section of the capsule prevents full range of motion • Altered flow of synovial fluid prevents fluid diffusion into and out of cartilage and causes compression and distention
  • 35. INJURIES TO FASCIAE AND BURSAE • Fasciae are connective tissues of the body arranged in sheaths that envelop muscles • Bursae are pockets of connective tissue lined with synovium in locations between muscles or between muscle or tendon and bone
  • 36. INJURIES TO NERVES, NERVE ROOTS, AND DURA MATER • Trauma to any soft tissue may lead to adhesive constriction of the nerve, nerve root, or dura mater • Irritation or entrapment of a nerve causes pain that radiates along the structures innervated by the nerve • Symptoms include altered sensation, motor weakness, and diminished reflexes
  • 37. INJURIES TO NERVES, NERVE ROOTS, AND DURA MATER (CONT.)
  • 38. INJURY TO TENDONS • Range from mild to complete tear • Tendinitis may develop from infection, direct injury or injury from repetitive motion • Tendons are injured when the stress placed on them is greater than the fibers can tolerate
  • 39. MUSCLE AND TENDON STRAINS • Categorized by the severity of injury • Grade I: minute tear of connective tissue and muscle fiber • Grade II: tear of a large portion of the contractile unit, with a segment still intact • Grade III: total rupture or loss of continuity of the contractile structure
  • 40. BLUNT TRAUMA • Can cause bleeding into the muscle belly; may lead to an inability to contract the muscle; may coagulate and calcify; results in myositis ossificans • Calcification prevents a normal and strong contraction of the muscle involved
  • 41. COMPARTMENT SYNDROME • Results from trauma to soft tissue caused by the unyielding structure of inert tissue • Causes: decreased compartment size, increased compartment content, or externally applied pressure • Edema causes increase in pressure in the compartment; pressure reduces capillary flow; muscle and nerves become ischemic • Symptoms may include pain, paresthesia, pale skin, and diminished pulse
  • 42. EVALUATION OF CONTRACTILE INJURIES • Patterns of function deal with pain and strength rather than excessive motion noted during evaluation of ligamentous injury • Strong and pain free • Strong and painful • Weak and painful • Weak and pain free
  • 43. SOFT-TISSUE HEALING AFTER TRAUMA • Disruption of circulatory and lymphatic systems; hemorrhage, fluid loss, cell death • Norepinephrine causes constriction of vessels; serotonin and platelets prolong vasoconstriction • Inflammation; release of histamines causes vasodilation and increased permeability of venules
  • 44. WOUND REPAIR • Inflammatory response prepares injured tissue • Granulation tissue develops in the wound space • Reepithelialization of the wound surface occurs; formation of basement membrane • Wound tensile strength is a result of deposition of collagen • Revascularization ensures survival of the new tissue
  • 45. WOUND REPAIR (CONT.) • Wound closure or contraction is the final phase of healing
  • 46. DISEASES OF SKELETAL MUSCLE • Skeletal muscle most abundant tissue in the body; accounts for 40% of body weight • Skeletal muscle performs dynamic and static work • Muscle atrophies in response to disuse and immobilization, and hypertrophies in response to increased stress
  • 47. MUSCULAR DYSTROPHY • A group of genetically determined myopathies characterized by progressive muscle weakness and degeneration • Muscle tissue is replaced by fat and fibrous connective tissue • Classified by the pattern of inheritance, age of onset, and distribution of muscular weakness
  • 48. DUCHENNE MUSCULAR DYSTROPHY • Most common and most severe form • Inherited as X-linked trait, thus affects only men • Muscle cells are deficient in the protein dystrophin; weakens the cell membrane and allows extracellular fluid to leak into the cell • Calf muscles enlarged, distal muscle involvement • Treatment includes corticosteroid therapy and preservation of function
  • 49. BECKER MUSCULAR DYSTROPHY • Milder form of inherited muscle degeneration • Genetic mutation leads to production of a reduced amount of an abnormal dystrophin protein and slower muscular degeneration • Calf hypertrophy prominent, often painful, with progressive loss of strength and ability to ambulate
  • 50. FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY • Rare inherited autosomal dominant trait that affects the muscles of the shoulder girdle and face • Facial muscles involved early with later involvement of scapular and upper arm musculature • Progresses slowly with periods of arrest and can ultimately involve more distal muscles
  • 51. MYASTHENIA GRAVIS • Chronic autoimmune disease; affects the neuromuscular function of voluntary muscles and characterized by profound muscle weakness and fatigability • Acetylcholine receptor antibodies produced that destroy or block acetylcholine receptors of the muscle end-plate of the neuromuscular junction
  • 52. MYASTHENIA GRAVIS (CONT.) • Anticholinesterase inhibitors, corticosteroids, intravenous immune globulin, plasmapheresis, and immunosuppressive agents may be used
  • 53. FIBROMYALGIA SYNDROME • Cause remains unknown • Characterized by widespread pain in all quadrants of the body; additional symptoms may include fatigue, insomnia, depression, and concentration problems • Treatment is multidimensional including exercise, counseling, and drug therapy to improve sleep, mood, and pain