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All the things > 18.4 Skeletal muscles and Neuromuscular stuff > Flashcards

18.4 Skeletal muscles and Neuromuscular stuff Flashcards

1
Q

What, generally, is dermatomyositis?

A

Idiopathic Inflammatory disorder of the skin and skeletal muscle

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2
Q

What is usually associated with dermatomyositis?

A

GI malignancies

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3
Q

What are the muscular features of dermatomyositis?

A
  • Bilateral proximal muscle weakness

- Distal involvement later in dz course

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4
Q

What are the skin features of dermatomyositis?

A
  • Rash of the upper eyelids (heliotrope rash)

- Red papules on the elbows, knuckles, and knees

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5
Q

Malar rash + bilateral proximal muscle weakness = ?

A

Dermatomyositis

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6
Q

What enzyme is classically elevated with dermatomyositis?

A

CK

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7
Q

What are the antibodies that are found with dermatomyositis?

A

ANA

Anti-Jo-1

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8
Q

What are the immune cells that cause the destruction in dermatomyositis? Where is the inflammation seen histologically?

A

CD4+ T cells

Perimysial inflammation

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9
Q

What is the treatment for dermatomyositis?

A

Corticosteroids

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10
Q

ANA +, malar rash, and Anti- Jo-1 = ?

A

Dermatomyositis

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11
Q

What is the CT layer that surrounds a single fascicle? Multiple?

A 
Single = perimysium
Multiple = Epimysium

(endomysium surrounds muscle fibers)

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12
Q

What, generally, is polymyositis? How does it compare to dermatomyositis?

A

Inflammatory disorder of the skeletal muscle that resembles dermatomyositis, but lacks skin involvement

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13
Q

What part of the muscles is inflamed with polymyositis? What are the immune cells that cause this?

A

Endomysial inflammation by CD8+ T cells

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14
Q

Match: (endomysial/ perimysial inflammation); (polymyositis/ dermatomyositis)

A
  • Polymyositis = endomysial

- Dermatomyositis = perimysial

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15
Q

What is the inheritance pattern of muscular dystrophies? What is the genetic defect with this?

A
  • XLR

- Deletions of dystrophin gene (for for Duchenne)

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16
Q

What is the pathological process that characterizes muscular dystrophy?

A

Replacement of skeletal muscles by adipose tissue

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17
Q

What is the function of dystrophin protein?

A

Anchors muscle cytoskeleton to the extracellular matrix

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18
Q

Why is muscular dystrophy often a spontaneous, as opposed to an inherited one?

A

Dystrophin gene is one of the largest in the human genome, and thus is more susceptible to damage

19
Q

What is the genetic defect in Becker’s vs Duchenne’s muscular dystrophy?

A 
Beckers = mutation
Duchennes = deletion
20
Q

How do kids with Duchenne muscular dystrophy usually present?

A

Proximal muscle weakness before 1 year of age

21
Q

What is the classic feature of Duchenne muscular dystrophy?

A

Calf pseudohypertrophy (2/2 fat deposition)

22
Q

What is the enzyme that is elevated with Duchenne muscular dystrophy?

A

CK

23
Q

What usually causes death with Duchenne muscular dystrophy?

A

Cardiac or respiratory failure

24
Q

What part of the heart is involved with Duchenne muscular dystrophy?

A

Myocardium

25
Q

Why is MG characterized by fatigable muscles?

A

Each time contraction occurs, less ACh is able to be release and compete with anti-nicotinic abs

26
Q

Are the antibodies with MG competitive or noncompetitive for ACh receptors?

A

Competitive

27
Q

Which gender is more commonly affected with MG?

A

Women

28
Q

What part of the body is classically affected with MG?

A

Eyes–ptosis and diplopia

29
Q

What is MG classically associated with and can be seen on CXR?

A

Thymic hyperplasia or thymoma

30
Q

What malignancy is classically associated with Lambert-Eaton syndrome?

A

small cell lung cancer

31
Q

Why does muscle strength improve with Eaton-Lambert syndrome?

A

Keep trying to force Ca in competes with antibody

32
Q

True or false: the eyes are classically affected with Eaton-Lambert syndrome

A

False– spared. MG is classically affected

33
Q

How do you differentiate MG and Eaton-Lambert syndrome clinically, besides history?

A

Anticholinesterase agents do not improve symptoms

34
Q

What is the most common benign soft tissue tumor in adults?

A

Lipomas

35
Q

What is the most common malignant soft tissue tumor in adults?

A

Liposarcomas

36
Q

What is the characteristic cell of liposarcomas?

A

Lipoblasts

37
Q

What is cardiac rhabdomyomas usually seen with?

A

Tuberous sclerosis

38
Q

What, generally, is a rhabdomyoma?

A

Benign tumor of skeletal muscle

39
Q

What, generally, is a rhabdomyosarcoma?

A

Malignant tumor of skeletal muscle

40
Q

What is the characteristic cell of rhabdomyosarcomas?

A

Rhabdomyoblast

41
Q

What is the most common site of a rhabdomyosarcoma? What is the classic presentation in young girls?

A

Head and neck most common

Vagina in young girls

42
Q

What is the most common malignant soft tissue tumor in children?

A

Rhabdomyosarcoma

43
Q

What is the stain that highlights rhabdomyosarcomas?

A

Desmin

44
Q

Young girl with a grape-like mass protruding from the vagina = ?

A

Rhabdomyosarcoma

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