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D & T exam 2 > Skeletal Muscle > Flashcards

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
how is spinal muscular atrophy inherited? what gene is messed up
autosomal recessive | -survival motor neuron 1
26
what happens to the cell in muscular dystrophy
internal nuclei | fibrofatty tissue
27
what protein is defected in Duchenne MD? what gene?
Dystrophin protein defect from abnormal gene at Xp21 ( deletion > frame shift or point mutation )
28
what are some clinical features of Duchenne MD?
normal at birth delayed walking pseudohypertrophy of calves increase CK
29
how do Duchenne MD patients die
respiratory failure, cardiac decompensation, lung infection
30
compare Becker MD with Duchenne MD
Becker MD less common, less severe, later onset
31
what part of the body does Limb Gridle MD occur? what does this cause
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
Limb Gridle MD is a defect in what
dystrophyn glycoprotein complex
33
Facioscapulohumeral MD has weakness where in the body? clinical significance
- facial weakness - wasting of upper arm and shoulder muscles - scapular bones look like wings when the arms are raised
34
Emery-Dreifuss MD onset
10-20;
35
what is the clinical triad for Emery-Dreifuss MD
- early humeroperoneal weakness - prominent contractures, ( elbows/ankles) - cardiomyopathy
36
muscle clinical signifiance for myotonic dystrophy
stiffness, difficulty releasing grip - precuss thenar eminence - weak foot dorsiflexion - weak intrinsic hand/wrist extension - facial muscle atrophy and ptosis
37
what are other clinical findings for myotonic dystrophy
cataracts endocrinopathy cardiomyopathy
38
what is wrong in the autosomal dominant myotonic dystrophy? protein and gene?
Dystrophila myotonia-protein kinase (DMPK) | CTG repeat expansion on 19q
39
what is the anticipation of mytonic dystrophy
onset at a younger age in succeeding generations
40
what are three types of hypotonic channelopathies
hyp0kalemic hyperkalmic normokalemic
41
what is the most common hypotonic channelopathy, tell me about it
hypokalemic - Ca channel mutation - attacks of flacid weakness
42
MOA of malignant hyperpyrexia
release Ca from SR - defective Ca channel and RyR1 - ATP depleted by muscle contraction - anaerobic metabolism
43
treatment for malignant hyperpyrexia
Dantrolene
44
clinical presentation for malignant hyperpyrexia
hypermetabolic state with tachypnea and general muscle contraction
45
central core disease how is inherited
autosomal dominant
46
what is wrong in central core disease
RyR1 defect
47
central core disease is a risk for
malignant hyperthermia
48
what happens in Nemaline Myopathy
- non-progressive hypotonia, weakness - proximal limb muslces - subsarcolemmal spindle-shaped particles fromZ band material
49
what happens to nuclues of centonuclear myopathy
central nuclei usually confined to type 1 fibers
50
lipid myopathies
accumulate lipid in myocytes - defects in carnitine transport system - defect in mitochondrial dehydrogenase enzyme system
51
mitochondrial myopathies
- defective mtDNA or nuclear DNA - ragged red fibers from aggregated mitochondira - parking lot for paracrystalline inlcusions
52
Dermatomyositis
skin rash precedes or begins with myositis ( grotten lesions and discoloration of eyelids with periobritl edema) - muscle weakness
53
Juvenline Dermatomyositis, how is it different from adult
GI involved causing abdominal pain
54
MOA of dermatomyositis
CD 4 t and b cells | - microvasculature attached by antibodies and compliment creating ischemia
55
Polymyositis
muscle weakness - autoantibodies against tRNA synthetases - cytotoxic CD8 t cells in endomysium destroy muscle
56
what lymphs are involved for DM, PM , IBM
DM: CD4 and B PM: CD 8 T IBM: CD8 T
57
which inflammatory myopathies respond to immunosupr
DM and PM
58
site of injury for inflammatory myopathies
DM: capillaries PM: endomysium myocyte IBM: endomysium myocyte
59
where do inflammatory myopathies begin on body
DM and PM: proximal | IBM: distal
60
intracellular deposits in inclusion body myosistis are similar to what other disease
Alzheimer's
61
what happens to the muslces of thyrotoxic mypopathy
acute or chronic proximal muscle weakness
62
clinicl symptom for thyrotoxic myopathy
exophthalmic ophthalmoplegia
63
clinical manifestations of hypothroidism myopathy
cramping/aching muscles slow movements slow reflexes proximal weaknes
64
Ethonal myopathy, what happens after binge drinking
acute rhabdomyolysis
65
name 3 drugs for drug induced myopathies
steroid ( proximal weakness, type 2 fibers) chloroquine ( proximal weakness) statins
66
what is ICU myopathy related to ? what does this do to muscles
corticosteroid therapy | - degraded myosin thick filaments
67
MOA for mysthenia gravis
immunemediated loss of acetylcholine receptors
68
myasthena gravis patients present with
drooping eyelid | diplopia
69
what is the muscle response for myasthenia gravis
decremental decrease in muscle response to repeated sitmulus
70
what is the tenselon test
short acting anticholenesterase
71
Lambert-Eaton myasthenic sydnrome
antibodies inhibit presynaptic calcium channel and block aceylcholine release
72
Lambert-Eaton myasthenic syndrome most likely comes from what disease
small cell carcinoma in lung
73
what is the muscle response forLambert-Eaton myasthenic syndrome
enhanced neurotransmission with repeitive stimulation

D & T exam 2 (27 decks)

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