Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Musculoskeletal System > Pathology II Myopathy > Flashcards

Pathology II Myopathy Flashcards

(68 cards)

Start Studying
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What diagnostic findings associated with Hoffmann disease?

A

Muscle biopsy

Will show atrophy of muscle cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

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

A

No reflex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Tx for Hoffman

A

Not available

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

From what condition do floppy infants generally die?

A

Respiratory failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

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

A

Grouped atrophy muscle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the two categories of muscular dystrophies?

A

Type I spinal cord atrophy

X-linked

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

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

A

UMN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

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

A

2/3 familial cases

X-linked

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Normal skeletal muscle biopsy

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

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

A

Immunoperoxidase stain

Useful in Duchenne’s diagnostic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Where is The gold stain seen in immunoperoxidase stain located ?

A

Periphery of the normal muscle fibers

If missing, suspect Duchenne’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Altered dystrophin

A

Becker’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

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

A

Muscles get replaced by fat and connective tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

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

A

Creatine kinase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
In Duchenne's, what is present at high levels and peaks early and then absent at later stages as muscle mass decreases?
Increased Creatine kinase in the first decade | Decreased CK Because muscles die
26
A teen presents with large calf and his muscle biopsy shows that fibers are increased and fat and connective tissue present
Pseudohypertrophy Not muscles per se that hypertrophy It is the tissue
27
In Duchenne's, chronic inflammation is present. What will be seen in the biopsy ?
Degeneration of muscle fibers along with some regeneration - scattered Hypertrophy of muscle fibers- larger contracted fibers Fibrosis Lymphocytes Macrophages?
28
Early development shows proximal muscle weakness
Duchenne's in early childhood
29
what other conditions cause death in pts with muscular dystrophy
Infection | Cardiac failure
30
A 60 year old have weakness, a slowly progressive disease. His brother may have had Sx
Becker's
31
Wheelchair by age 10
Duchenne's
32
Most common form of dystrophy In Adults
Myotonic dystrophy
33
A patient shakes hand but can't let go because of impaired muscle relaxation
Myotonic dystrophy
34
What happens to the muscle fibers in DM
Atrophy of type I fibers | Hypertrophy of type II fibers
35
Duchenne's vs DM on biopsy
Muscle fibers are not necrotic or regenerated as much in DM
36
A patient presents with stiffness and unable to relax grip from handshake, facial and jaw muscles are affected most, and he has cataracts
Multi system affect The patient has DM
37
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
Clinical signs of myotonic dystrophy
38
How to determine gyencomastia in pts with DM?
Palpation of breast tissue
39
What is a physical exam for DM ?
Percussion of thener muscles
40
A 60 year old pt's thumb moves sharply into opposition and adduction and slowly returns to initial position on percussion of thener muscles
Pt has myotonic dystrophy
41
A 6 month old baby has a tented, inverted V shape of upper lip, and pyramidal lip shape
Acquired disease in rare form | Infant has Myotonic dystrophy
42
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
Myotonic dystrophy
43
What is a major differential for Myotonic vs muscular dystrophies ?
Size of the fibers are homogenous
44
What are toxic myopathies?
Thyrotoxic myopathy Ethanol myopathy Drug-induced myopathy
45
A patient presents with proximal muscle weakness, exophthalmos, muscle cramps, and slow movement and reflexes
Thyrotoxic myopathy - a thyroid dysfunction | Males are 4x affected
46
Which clinical Sx presents before exophthalmos in thyrotoxic myopathy ?
Proximal muscle weakness
47
Grave's disease
``` Increased production of thyroid hormones T3 and T4 Metabolic rate goes up Heat Intolerant Fat and muscle breakdown Weight loss ```
48
Is thyrotoxic myopathy similar to grave's disease?
Yes or no?
49
An intoxicated pt presents was taken into ER. His urine results show that it is red
Ethanol myopathy, specifically acute myopathy Myoglobinemia is suspected because of urine result The increasing level of myoglobin indicates
50
What results from binge drinking ?
Acute myopathy | Renal failure
51
What is rhabdomyolysis?
Cause muscle breakdown | Associated with acute myopathy from binge drinking
52
What class of drugs induce myopathy?
Steroids | Chloroquine (anti malarial)
53
How do steroids affect muscles?
Cause them to breakdown Results in muscle weakness Type 2 muscle fibers atrophy
54
What is the best defined autoimmune disease?
Myasthenia gravis Ab are made against Ach rcpts which are eventually degraded by cells Efferent discharge (parasympathetic) usually release Ach
55
What occurs in the thymus gland in people with MG?
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
What is the first sign of MG?
``` Extraocular muscles -Ptosis -Diplopia- double vision Generalized weakness Respiratory compromised Low nerve conduction with repetitive stimulation ```
57
A pt. presents with ptosis, Diplopia, and weakness overall
MG if tx with anti-Acholinesterase improve condition
58
A major cause of death in pts with MG
Respiratory compromise
59
What is the expected survival for MG pts?
95% 5 year survival
60
What EMG finding will indicate MG?
Repetitively stimulate a nerve in MG and the velocity of the conduction will decrease
61
What is the rationale for tx MG with anti-Achase?
Increase amnt of Ach - allowing Ach linger and bind to rcpts
62
What are the other two drugs used to tx MG
Prednisone- glucocorticoid | Plasmapheresis - remove Ab from the circulation
63
Lambert Eden syndrome
Disease of neuromuscular junction Develops as paraneoplastic process Proximal muscle weakness Autoimmunity to ca2+ may be responsible
64
What is the paraneoplastic process?
Lung cancer | Small cell carcinoma of lung (60%)
65
How does EMG finding in Lambert Eden distinguish from MG?
The velocity of conduction is increased with repetitive stimulation
66
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?
Can't release Ach because Ca2+ channels blocked?
67
In what disease will Chemo and radiation best extend the life to one year in pts
Lambert Eden
68
What's the rationale for increased EMG in lambert Eden ?
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)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions