Skeletal Muscle Flashcards

1
Q

myopathy

A

disorder of muscle

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2
Q

segmental muscle necrosis

A

destruction of a portion of the myocyte length

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3
Q

how does muscle regeneration occur

A

satellite cells reconstitute destroyed muscles

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4
Q

when does fiber hypertrophy occur

A

response to an increase in load

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5
Q

myotonia

A

tonic spasm of one or more muscles

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6
Q

hypotonia

A

deficient tone or tension

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7
Q

arthrogryposis

A

fixation joints in an extended or flexed position

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8
Q

what is creatine phosphokinase (CK; CPK)

A

enzyme highly concentrated in muscle and brain

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9
Q

Gower’s sign

A

indicates weakness of the proximal muscles

- uses hands and arms to walk up form squatting position

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10
Q

Myofibril

A

repeating units of sarcomeres

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11
Q

What defines a sarcomere

A

Z line to Z line

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12
Q

what makes a thin filaments in the muscle

A

actin

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13
Q

what makes up thick filaments in the muscle

A

myosin

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14
Q

what is the Z band primarily made up of

A

actin

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15
Q

what is the cell membrane of a muscle cell called

A

sarcolemma

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16
Q

what is the cytoplasm of a muscle cell called

A

sarcoplasm

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17
Q

when a muscle cell contracts what regions contract, lengthen.

A

I band shortens

A band unchanged

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18
Q

acronym of muscle fibers

A

One slow fat red ox

1, lipid rich, high myoglobin, oxidative

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19
Q

which muscle fiber is for sustained use? sudden use?

A

fiber 1: sustained

fiber 2: sudden

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20
Q

grouped atrophy

A

loss of a motor neuron leads to atrophy of associated muscle

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21
Q

fiber type grouping

A

neuron/axon drop out leads to larger motor units

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22
Q

Denervation atrophy is disorder of what? breakdown of what?

A
  • motor neurons

breakdown of myosin and actin

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23
Q

what is wrong in spinal muscular atrophy

A

progressive destruction of anterior horn cells and cranial nerve motor neurons

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24
Q

another name for spinal muscular atrophy

A

infantile motor neuron disease

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25
Q

how is spinal muscular atrophy inherited? what gene is messed up

A

autosomal recessive

-survival motor neuron 1

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26
Q

what happens to the cell in muscular dystrophy

A

internal nuclei

fibrofatty tissue

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27
Q

what protein is defected in Duchenne MD? what gene?

A

Dystrophin protein defect from abnormal gene at Xp21 ( deletion > frame shift or point mutation )

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28
Q

what are some clinical features of Duchenne MD?

A

normal at birth
delayed walking
pseudohypertrophy of calves
increase CK

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29
Q

how do Duchenne MD patients die

A

respiratory failure, cardiac decompensation, lung infection

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30
Q

compare Becker MD with Duchenne MD

A

Becker MD less common, less severe, later onset

31
Q

what part of the body does Limb Gridle MD occur? what does this cause

A

proximal muscles

  • waddling gait because of weak hip and leg muscles
  • trouble getting out of chairs or climbing stairs
  • reaching over head hard
  • holding arms outstretched
32
Q

Limb Gridle MD is a defect in what

A

dystrophyn glycoprotein complex

33
Q

Facioscapulohumeral MD has weakness where in the body? clinical significance

A
  • facial weakness
  • wasting of upper arm and shoulder muscles
  • scapular bones look like wings when the arms are raised
34
Q

Emery-Dreifuss MD onset

A

10-20;

35
Q

what is the clinical triad for Emery-Dreifuss MD

A
  • early humeroperoneal weakness
  • prominent contractures, ( elbows/ankles)
  • cardiomyopathy
36
Q

muscle clinical signifiance for myotonic dystrophy

A

stiffness, difficulty releasing grip

  • precuss thenar eminence
  • weak foot dorsiflexion
  • weak intrinsic hand/wrist extension
  • facial muscle atrophy and ptosis
37
Q

what are other clinical findings for myotonic dystrophy

A

cataracts
endocrinopathy
cardiomyopathy

38
Q

what is wrong in the autosomal dominant myotonic dystrophy? protein and gene?

A

Dystrophila myotonia-protein kinase (DMPK)

CTG repeat expansion on 19q

39
Q

what is the anticipation of mytonic dystrophy

A

onset at a younger age in succeeding generations

40
Q

what are three types of hypotonic channelopathies

A

hyp0kalemic
hyperkalmic
normokalemic

41
Q

what is the most common hypotonic channelopathy, tell me about it

A

hypokalemic

  • Ca channel mutation
  • attacks of flacid weakness
42
Q

MOA of malignant hyperpyrexia

A

release Ca from SR

  • defective Ca channel and RyR1
  • ATP depleted by muscle contraction
  • anaerobic metabolism
43
Q

treatment for malignant hyperpyrexia

A

Dantrolene

44
Q

clinical presentation for malignant hyperpyrexia

A

hypermetabolic state with tachypnea and general muscle contraction

45
Q

central core disease how is inherited

A

autosomal dominant

46
Q

what is wrong in central core disease

A

RyR1 defect

47
Q

central core disease is a risk for

A

malignant hyperthermia

48
Q

what happens in Nemaline Myopathy

A
  • non-progressive hypotonia, weakness
  • proximal limb muslces
  • subsarcolemmal spindle-shaped particles fromZ band material
49
Q

what happens to nuclues of centonuclear myopathy

A

central nuclei usually confined to type 1 fibers

50
Q

lipid myopathies

A

accumulate lipid in myocytes

  • defects in carnitine transport system
  • defect in mitochondrial dehydrogenase enzyme system
51
Q

mitochondrial myopathies

A
  • defective mtDNA or nuclear DNA
  • ragged red fibers from aggregated mitochondira
  • parking lot for paracrystalline inlcusions
52
Q

Dermatomyositis

A

skin rash precedes or begins with myositis ( grotten lesions and discoloration of eyelids with periobritl edema)
- muscle weakness

53
Q

Juvenline Dermatomyositis, how is it different from adult

A

GI involved causing abdominal pain

54
Q

MOA of dermatomyositis

A

CD 4 t and b cells

- microvasculature attached by antibodies and compliment creating ischemia

55
Q

Polymyositis

A

muscle weakness

  • autoantibodies against tRNA synthetases
  • cytotoxic CD8 t cells in endomysium destroy muscle
56
Q

what lymphs are involved for DM, PM , IBM

A

DM: CD4 and B
PM: CD 8 T
IBM: CD8 T

57
Q

which inflammatory myopathies respond to immunosupr

A

DM and PM

58
Q

site of injury for inflammatory myopathies

A

DM: capillaries
PM: endomysium myocyte
IBM: endomysium myocyte

59
Q

where do inflammatory myopathies begin on body

A

DM and PM: proximal

IBM: distal

60
Q

intracellular deposits in inclusion body myosistis are similar to what other disease

A

Alzheimer’s

61
Q

what happens to the muslces of thyrotoxic mypopathy

A

acute or chronic proximal muscle weakness

62
Q

clinicl symptom for thyrotoxic myopathy

A

exophthalmic ophthalmoplegia

63
Q

clinical manifestations of hypothroidism myopathy

A

cramping/aching muscles
slow movements
slow reflexes
proximal weaknes

64
Q

Ethonal myopathy, what happens after binge drinking

A

acute rhabdomyolysis

65
Q

name 3 drugs for drug induced myopathies

A

steroid ( proximal weakness, type 2 fibers)
chloroquine ( proximal weakness)
statins

66
Q

what is ICU myopathy related to ? what does this do to muscles

A

corticosteroid therapy

- degraded myosin thick filaments

67
Q

MOA for mysthenia gravis

A

immunemediated loss of acetylcholine receptors

68
Q

myasthena gravis patients present with

A

drooping eyelid

diplopia

69
Q

what is the muscle response for myasthenia gravis

A

decremental decrease in muscle response to repeated sitmulus

70
Q

what is the tenselon test

A

short acting anticholenesterase

71
Q

Lambert-Eaton myasthenic sydnrome

A

antibodies inhibit presynaptic calcium channel and block aceylcholine release

72
Q

Lambert-Eaton myasthenic syndrome most likely comes from what disease

A

small cell carcinoma in lung

73
Q

what is the muscle response forLambert-Eaton myasthenic syndrome

A

enhanced neurotransmission with repeitive stimulation