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NMSP - Exam 2 > Diseases of Skeletal Muscle > Flashcards

Diseases of Skeletal Muscle Flashcards

1
Q

What are 3 Inflammatory Myopathies?

A
  1. Dermatomyositis
  2. Polymyositis
  3. Inclusion Body Myositis
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2
Q

What histologic change is seen with Dermatomyositis?

A

Perifascicular Atrophy

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3
Q

Generally, what are the signs of Dermatomyositis?

A

Skin changes

Proximal bilateral muscle weakness

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4
Q

What unique skin changes and where are seen with Dermatomyositis?

A
  • Lilac/Heliotrope discoloration of the upper eyelid

- Periorbital edema

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5
Q

Lilac/Heliotrope discoloration of upper eyelids, periorbital edema and telangiectasias can be seen with?

A

Dermatomyositis

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6
Q

What specific lesions can present with Dermatomyositis?

A

Grotton Lesions

- red/dusky patches over knuckles, elbows, knees

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7
Q

Dermatomyositis involves proximal bilateral muscle weakness. What 3 other things can be present?

A

ILD
Dysphagia
Cardiac involvement

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8
Q

What autoantibodies with Dermatomyositis cause the heliotrope rash and grotton lesions?

A

Anti-Mi2

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9
Q

What autoantibodies with Dermatomyositis cause the ILD?

A

Anti-Jo1

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10
Q

What autoantibodies with Dermatomyositis can cause the juvenile cases and when does that present?

A

7 years old

= Anti-P155/140

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11
Q

What are the 2 signs of Juvenile Dermatomyositis?

A

Calcinosis

Lipodystrophy

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12
Q

Patients with Dermatomyositis have an increased risk of?

A

Visceral (occult) cancers

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13
Q

What are the symptoms of Dermatomyositis?

A
  • Lilac/heliotrope discoloration of upper eyelids
  • Periorbital edema
  • Telangiectasias
  • Grotton Lesions
  • Proximal Bilateral muscle weakness
  • Dysphagia
  • ILD
  • Cardiac involvement
  • Occult malignancies
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14
Q

What mediates Polymyositis?

A

CD8 T cells

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15
Q

What histo change is seen with Polymyositis?

A

Endomysial mononuclear infiltrate

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16
Q

What are the symptoms of Polymyositis?

A

Proximal bilateral muscle weakness and pain

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17
Q

Proximal bilateral muscle weakness and pain

A

Polymyositis

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18
Q

What are the symptoms of Inclusion Body Myositis?

A

Asymmetric progressive weakness of quadriceps, wrist and finger flexors

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19
Q

Asymmetric progressive weakness of quadriceps, wrist and finger flexors

A

Inclusion body myositis

20
Q

What muscles are weak with Inclusion Body Myositis?

A

Quadriceps

Wrist and finger flexors

21
Q

What histo change will be seen with Inclusion Body Myositis?

A

Rimmed vacuoles with reddish granules around periphery

22
Q

Rimmed vacuoles with reddish granules around periphery

A

Inclusion Body Myositis

23
Q

Which of the Inflammatory Myopathies does NOT respond to steroids or immune suppression?

A

Inclusion Body Myositis

24
Q

What 2 types of drugs can cause toxic myopathies?

A

Statin drugs

Anti-malarial drugs

25
Anti-malarial drugs can cause what things to be affected with its myopathy?
Type 1 fibers | Cardiac muscle
26
ICU myopathy involves a ____ deficit
Myosin deficit
27
ICU myopathy
Illness with corticosteroid treatment
28
How is Central Core Disease inherited?
AD
29
What mutation is present with Central Core Disease?
RYR1 mutation
30
RYR1 mutation
Central Core Disease
31
What are the general symptoms of Central Core Disease?
Malignant Hyperthermia and myopathy
32
Increased HR, RR, muscle spasms and hyperpyrexia may be?
Central Core Disease | -- malignant hyperthermia and myopathy
33
What triggers Central Core Disease and then what happens?
Anesthetics - -> increased efflux of calcium - -> tetany and heat production
34
What triggers Central Core Disease and what happens?
Anesthetics - -> increased efflux of calcium - -> tetany and heat production
35
How is Spinal Muscular Atrophy inherited?
AR
36
What is lost with Spinal Muscular Atrophy?
Loss of spinal cord anterior horn cells => loss of motor neurons
37
Loss of spinal cord anterior horn cells that causes loss of motor neurons
Spinal Muscular Atrophy
38
What is a sign of Spinal Muscular Atrophy?
FLOPPY BABY = generalized hypotonia
39
What gene on what chromosome is deficient with Spinal Muscular Atrophy?
SMN1 on chr. 5
40
SMN1 on chr. 5 mutation
Spinal Muscular Atrophy -- floppy baby (generalized hypotonia)
41
What myofibers will be seen histologically with Spinal Muscular Atrophy?
Atrophic + normal + hypertrophied myofibers
42
What is the most common type of Spinal Muscular Atrophy?
Wernig-Hoffman
43
What will be the signs of Wernig-Hoffman (spinal muscular atrophy)?
Muscle weakness - -> chewing, swallowing and breathing difficulties - -> death by 3 yo
44
What will be the signs of Wernig-Hoffman?
Muscle weakness - -> chewing, swallowing and breathing difficulties - -> fatal by 3 yo
45
What is an Ion Channel Myopathy?
Central Core Disease | -- malignant hyperthermia and myopathy

NMSP - Exam 2 (29 decks)

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