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SKELETAL MUSCLE PATHOLOGY

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SKELETAL MUSCLE PATHOLOGY Inclusion body myositis IBM causes progressive weakness of the muscles of the wrists and fingers, the muscles of the front of the thigh and ... – PowerPoint PPT presentation

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Title: SKELETAL MUSCLE PATHOLOGY


1
SKELETAL MUSCLE PATHOLOGY
2
Normal skeletal muscle
3
Pathophysiological changes of muscles
  • Atrophy and hypertrophy of muscles

4
Atrophy and hypertrophy of muscles
5
Muscle atrophies
  • Muscle atrophy is defined as a decrease in the
    mass of the muscle
  • Physiologically Muscle mass, muscle strength,
    and bone density decrease in the elderly
  • Disuse atrophy of muscles can occur after
    prolonged immobility such as extended bed-rest,
    or having a body part in a cast.
  • This type of atrophy can usually be reversed with
    exercise.

6
Disuse Atrophy Of Muscles
7
  • There are many diseases and conditions which
    cause atrophy of muscle mass.
  • For example diseases such as cancer and AIDS
    induce a body wasting syndrome called "cachexia
  • Other conditions which can induce skeletal muscle
    atrophy are congestive heart failure and liver
    disease.

8
Severe generalized muscle atrophy Body wasting
syndrome called "Cachexia
9
Muscle hypertrophy
  • Muscle hypertrophy is an increase in the size of
    a muscle
  • Hypertrophy is an increase in mass of a muscle
    that can be induced by a number of stimuli. The
    most familiar of these is exercise.
  • Pathologically in Acromegaly disease there is
    pathological muscle hypertrophy affecting mainly
    the type (1) skeletal fibers

10
Exercise Muscle Hypertrophy
Acromegaly Disease
11
Muscle Diseases
12
Classification of Muscle Disease
  • Inflammatory Myopathies
  • Infectious Myositis
  • Non-Infectious Myositis (Auto-immune )
  • 1-Polymyositis
  • 2-Dermatomyositis
  • 3-Inclusion body myositis
  • Diseases of the Neuromuscular Junction
  • -Myasthenia Gravis
  • -Lambert-Eaton Syndrome

13
  • Dystrophies
  • Duchennes Muscular Dystrophy
  • Beckers Muscular Dystrophy
  • Myotonic Dystrophy
  • Metabolic Myopathies
  • -Glycogen Storage Diseases
  • -Mitochondrial Myopathies
  • Toxic Myopathies

14
Inflammatory Myopathies
15
Inflammatory myositis
  • Polymyositis
  • Dermatomyositis
  • Inclusion body myositis
  • Are of auto-immune origin

16
Polymyositis (PM)
  • It means "inflammation of many muscles") it is a
    type of chronic inflammation of the muscles
    possibly due to autoimmune causes

17
  • Clinical presentation
  • Adults
  • Bilateral proximal muscle weakness
  • Microscopic
  • Endomysial lymphocytic inflammation
  • Skeletal muscle fiber degeneration and
    regeneration

18
POLYMYOSITIS mostly affects the muscles of the
hips and thighs, the upper arms, the top part of
the back, the shoulder area and the neck
19
Signs and symptoms

  • Symptoms include pain with marked weakness and
    loss of muscle mass in the proximal musculature
    particularly in the shoulder and pelvic girdle.
  • The hip extensors are often severely affected
    leading to particular difficulty in ascending
    stairs and rising from a seated position.
  • Dysphagia (difficulty in swallowing) occurs in
    1/3 of patients.
  • Low grade fever and peripheral lymphadenopathy
    may be present.

20
Muscle weakness can cause difficulty in many
daily activities
21
Diagnosis
  • History and physical examination
  • Elevation of creatine kinase
  • Electromyograph (EMG) alteration
  • Positive muscle biopsy

22
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23
Endomysial lymphocytic inflammation
Polymyositis
24
Dermatomyositis
  • Dermatomyositis (DM) is a connective-tissue
    disease related to polymyositis (PM) that is
    characterized by inflammation of the muscles and
    the skin.
  • The disease may also affect the joints, the
    esophagus, the lungs, and, less commonly, the
    heart.

25
Dermatomyositis
  • Clinical presentation
  • Children and adults
  • Bilateral proximal muscle weakness
  • Skin rashes (upper eyelids)
  • Peri-orbital edema
  • Microscopic
  • Perimysial and vascular lymphocytic inflammation
  • Skeletal muscle fiber degeneration and
    regeneration

26
  • Grotton lesions scaly erythematous eruptions or
    red patches overlying the knuckles, elbows, and
    knees
  • X-ray findings sometimes include dystrophic
    calcifications in the muscles, and patients may
    or may not notice small calcium deposits under
    the skin

27
Gottron's papules Discrete erythematous papules
overlying the metacarpal and interphalangeal
joints in a patient with juvenile dermatomyositis
28
Gottron's sign. Confluent macular erythema with
scale confined to the skin overlying the patellae
in a juvenile dermatomyositis
29
X-Ray of the knee in a patient with
dermatomyositis
30
Clinical presentation
  • The main symptoms include skin rash and symmetric
    muscle weakness which may be accompanied by pain.
  • The heliotrope or "lilac" rash is a violaceous
    eruption on the upper eyelids and in rare cases
    on the lower eyelids as well, often with itching
    and swelling

31
Eruption is associated with peri-orbital edema
and telangiectasias of the both eyelids.
32
Diagnosis
  • The diagnosis of dermatomyositis is usually
    confirmed by muscle biopsy, EMG and blood tests.
  • Liver enzymes, specifically creatin phosphokinase
    (CPK), are the major tool in assessing the
    progress of the disease and/or the efficacy of
    treatment

33
Perimysial and vascular lymphocytic inflammation
Dermatomyositis
34
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35
Inclusion body myositis
  • IBM causes progressive weakness of the muscles of
    the wrists and fingers, the muscles of the front
    of the thigh and the muscles that lift the front
    of the foot.
  • IBM is generally a slowly progressive disease,
    and life expectancy isnt significantly affected.
    Most people with IBM remain able to walk,
    although they may require a cane or wheelchair
    for long distances.

36
  • Clinical presentation
  • Adultsgt age 50
  • Asymmetrical distal muscle weakness
  • Microscopically
  • Cytoplasmic vacuoles with basophilic granules and
    amyloid

37
The first muscles affected in inclusion-body
myositis are usually those of the wrists and
fingers, and the muscles at the front of the
thigh. The muscles that lift the front of the
foot also may be affected
38
Diagnosis
  • Elevated creatine kinase CK levels
  • Electromyography (EMG) studies usually display
    abnormalities.
  • Muscle biopsy display several findings including
    inflammatory cells invading muscle cells,
    vacuolar degeneration, inclusions or plaques of
    abnormal proteins.

39
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40
Cytoplasmic vacuoles
Inclusion body myositis
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