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Musculoskeletal System > Pathology II Myopathy > Flashcards

Pathology II Myopathy Flashcards

1
Q

Denervation atrophy

A

Any process that affects anterior horn cells or axon in PNS
Spinal injury - Alpha motor neuron is not innervating the muscle due to spinal injury , therefore muscles will atrophy
LMN muscle atrophy

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2
Q

A 1 year old baby cannot sit up and show muscle weakness. Considered to have flaccid paralysis in the trunk and limbs and lack of sucking ability . Has respiratory failure

A

Werdnig-Hoffman autosomal recessive disease
Congenital disease -early onset at birth to 4months
Death within 3 years
floppy baby syndrome is presented
Muscles of the mouth don’t work

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3
Q

What diagnostic findings associated with Hoffmann disease?

A

Muscle biopsy

Will show atrophy of muscle cell

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4
Q

Type I spinal motor atrophy

A

Degeneration of the cells in the anterior horn of the spinal cord and the motor nuclei in the brainstem
Afferent neurons don’t synapse to anything

Group of inherited disorders

  • beginning in childhood
  • progressive weakness and wasting of muscles
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5
Q

What kind of stretch reflex result will be present in spinal motor atrophy?

A

No reflex

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6
Q

Tx for Hoffman

A

Not available

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7
Q

From what condition do floppy infants generally die?

A

Respiratory failure

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8
Q

On a biopsy, what is the name of thedistinctive atrophy seen for werdnig-Hoffman disease?

A

Grouped atrophy muscle

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9
Q

Grouped atrophy

A

Nuclei of the muscle fibers are in the periphery
Not all muscles atrophy
Atrophy occurs from disuse of the muscles

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10
Q

What are the two categories of muscular dystrophies?

A

Type I spinal cord atrophy

X-linked

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11
Q

What type of motor lesion don’t cause muscle atrophy?

A

UMN

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12
Q

A 16 year presents with muscle biopsy that shows no dystrophin, muscle fiber size variations, nuclei centrally located, increased connective tissue

A

Duchenne’s

Which is a more severe and common disease

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13
Q

A 16 year old has trouble contracting muscles. The disease was presented in his grandfather.

A

2/3 familial cases

X-linked

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14
Q

What happens to the muscle fibers in Duchenne’s?

A

Regeneration of muscle fibers
Turnover of fibers occur because they become necrotic and phagocytosis
Turnover rate exceed regeneration =overall decrease in muscle fibers

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15
Q

Normal skeletal muscle biopsy

A

Cross section view reveals multiple nuclei of the fiber are at the periphery of the muscle fiber

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16
Q

What is found on a muscle biopsy of Duchenne’s and beckers

A 
Variation of muscle fiber size
Increased endomysial connective tissue -bluish area 
Heterogenous tissue
Nuclei centralize 
Regeneration of fibers
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17
Q

What does western blotting reveal for Duchenne’s vs Becker’s

A

Dystrophin is absent in Duchenne’s and low amount in Becker’s

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18
Q

What uses antibody to the muscle dystrophin to view gold stain?

A

Immunoperoxidase stain

Useful in Duchenne’s diagnostic

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19
Q

Where is The gold stain seen in immunoperoxidase stain located ?

A

Periphery of the normal muscle fibers

If missing, suspect Duchenne’s

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20
Q

The gold stain is present in small amounts in the patient

A

Suspect Becker

Some muscle cells will have low amnt of dystrophin

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21
Q

Altered dystrophin

A

Becker’s

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22
Q

A teen complaints of muscle weakness in pelvic and it also extends to the shoulder

A

A Major early complaint of people with Duchenne’s

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23
Q

At later stages of DMD, BMD, what happens to the muscle fibers?

A

Muscles get replaced by fat and connective tissue

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24
Q

What is the protein found in serum that tells you whether or not the muscles are going necrosis?

A

Creatine kinase

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25
Q

In Duchenne’s, what is present at high levels and peaks early and then absent at later stages as muscle mass decreases?

A

Increased Creatine kinase in the first decade

Decreased CK Because muscles die

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26
Q

A teen presents with large calf and his muscle biopsy shows that fibers are increased and fat and connective tissue present

A

Pseudohypertrophy

Not muscles per se that hypertrophy
It is the tissue

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27
Q

In Duchenne’s, chronic inflammation is present. What will be seen in the biopsy ?

A

Degeneration of muscle fibers along with some regeneration - scattered
Hypertrophy of muscle fibers- larger contracted fibers
Fibrosis
Lymphocytes
Macrophages?

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28
Q

Early development shows proximal muscle weakness

A

Duchenne’s in early childhood

29
Q

what other conditions cause death in pts with muscular dystrophy

A

Infection

Cardiac failure

30
Q

A 60 year old have weakness, a slowly progressive disease. His brother may have had Sx

A

Becker’s

31
Q

Wheelchair by age 10

A

Duchenne’s

32
Q

Most common form of dystrophy In Adults

A

Myotonic dystrophy

33
Q

A patient shakes hand but can’t let go because of impaired muscle relaxation

A

Myotonic dystrophy

34
Q

What happens to the muscle fibers in DM

A

Atrophy of type I fibers

Hypertrophy of type II fibers

35
Q

Duchenne’s vs DM on biopsy

A

Muscle fibers are not necrotic or regenerated as much in DM

36
Q

A patient presents with stiffness and unable to relax grip from handshake, facial and jaw muscles are affected most, and he has cataracts

A

Multi system affect

The patient has DM

37
Q

A 40 year old presents with frontal balding, hatchet facies due to atrophy of temporis muscle, ptosis, drooping mouth due to facial weakness, cataracts, wasting of sternlcleidomastoid, and gynecomastia

A

Clinical signs of myotonic dystrophy

38
Q

How to determine gyencomastia in pts with DM?

A

Palpation of breast tissue

39
Q

What is a physical exam for DM ?

A

Percussion of thener muscles

40
Q

A 60 year old pt’s thumb moves sharply into opposition and adduction and slowly returns to initial position on percussion of thener muscles

A

Pt has myotonic dystrophy

41
Q

A 6 month old baby has a tented, inverted V shape of upper lip, and pyramidal lip shape

A

Acquired disease in rare form

Infant has Myotonic dystrophy

42
Q

Pt’s lab results show presence of central nuclei in the muscle fibers in cross section (The centralized nuclei are arranged in longitudinal fashion) along with variation in fiber size, fibrosis

A

Myotonic dystrophy

43
Q

What is a major differential for Myotonic vs muscular dystrophies ?

A

Size of the fibers are homogenous

44
Q

What are toxic myopathies?

A

Thyrotoxic myopathy
Ethanol myopathy
Drug-induced myopathy

45
Q

A patient presents with proximal muscle weakness, exophthalmos, muscle cramps, and slow movement and reflexes

A

Thyrotoxic myopathy - a thyroid dysfunction

Males are 4x affected

46
Q

Which clinical Sx presents before exophthalmos in thyrotoxic myopathy ?

A

Proximal muscle weakness

47
Q

Grave’s disease

A 
Increased production of thyroid hormones T3 and T4 
Metabolic rate goes up 
Heat Intolerant
Fat and muscle breakdown 
Weight loss
48
Q

Is thyrotoxic myopathy similar to grave’s disease?

A

Yes or no?

49
Q

An intoxicated pt presents was taken into ER. His urine results show that it is red

A

Ethanol myopathy, specifically acute myopathy
Myoglobinemia is suspected because of urine result
The increasing level of myoglobin indicates

50
Q

What results from binge drinking ?

A

Acute myopathy

Renal failure

51
Q

What is rhabdomyolysis?

A

Cause muscle breakdown

Associated with acute myopathy from binge drinking

52
Q

What class of drugs induce myopathy?

A

Steroids

Chloroquine (anti malarial)

53
Q

How do steroids affect muscles?

A

Cause them to breakdown
Results in muscle weakness
Type 2 muscle fibers atrophy

54
Q

What is the best defined autoimmune disease?

A

Myasthenia gravis
Ab are made against Ach rcpts which are eventually degraded by cells
Efferent discharge (parasympathetic) usually release Ach

55
Q

What occurs in the thymus gland in people with MG?

A
  1. Thymic gland undergoes hyperplasia (65%)
    Produce Ab
    Remove the thymus to tx MG-will cure MG only if the source of Ab production is from the thymus
  2. Thymic tumor
56
Q

What is the first sign of MG?

A 
Extraocular muscles
-Ptosis 
-Diplopia- double vision 
Generalized weakness
Respiratory compromised 
Low nerve conduction with repetitive stimulation
57
Q

A pt. presents with ptosis, Diplopia, and weakness overall

A

MG if tx with anti-Acholinesterase improve condition

58
Q

A major cause of death in pts with MG

A

Respiratory compromise

59
Q

What is the expected survival for MG pts?

A

95% 5 year survival

60
Q

What EMG finding will indicate MG?

A

Repetitively stimulate a nerve in MG and the velocity of the conduction will decrease

61
Q

What is the rationale for tx MG with anti-Achase?

A

Increase amnt of Ach - allowing Ach linger and bind to rcpts

62
Q

What are the other two drugs used to tx MG

A

Prednisone- glucocorticoid

Plasmapheresis - remove Ab from the circulation

63
Q

Lambert Eden syndrome

A

Disease of neuromuscular junction
Develops as paraneoplastic process
Proximal muscle weakness
Autoimmunity to ca2+ may be responsible

64
Q

What is the paraneoplastic process?

A

Lung cancer

Small cell carcinoma of lung (60%)

65
Q

How does EMG finding in Lambert Eden distinguish from MG?

A

The velocity of conduction is increased with repetitive stimulation

66
Q

A pt presents with proximal muscle weakness and known to have autonomic dysfunction. No sign of improvement shown with anticholinerase. What is mechanism of the disease?

A

Can’t release Ach because Ca2+ channels blocked?

67
Q

In what disease will Chemo and radiation best extend the life to one year in pts

A

Lambert Eden

68
Q

What’s the rationale for increased EMG in lambert Eden ?

A

Initially no change in nerve conduction
Stimulation of 10s or more will lead to calcium being let into cell allowing the release of Ach, perhaps something to do with the build up of Ach in the terminus, which eventually going to be released when the electrical stimuli overrides it

Musculoskeletal System (27 decks)

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