Pathophysiology One Flashcards

1
Q

what are the 2 classifications of myopathy?

A
  • heredity
  • acquired
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2
Q

what are some types of hereditary myopathies?
ccmmmm

A
  • congenital myopathy
  • muscular dystrophies
  • myotonia and channelopathies
  • primary metabolic myopathies
  • mitochondrial myopathies
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3
Q

what are some types of acquired myopathies

A
  • inflammatory myopathies
  • infectious myopathies
  • endocrine myopathies
  • secondary metabolic myopathies
  • drug induced and toxic myopathies
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4
Q

_________ is a broad term for any muscle disorder present at birth

A
  • congenital myopathy
  • primarily affects skeletal muscle fibers and causes muscular weakness and/or hypotonia
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5
Q

_____________ is one of a group of muscle diseases know a the inflammatory myopathies, which are characterized by chronic, progressive muscle inflammation accompanies by muscle weakness

A

inclusion body myositis (IBM)

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6
Q

A positive gowers sign tells you what?

A

there is proximal muscle weakness

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7
Q

what 2 types of myopathies are characterized by loss of muscle mass?

A
  • atrophic myopathies (loss of CSA secondary to underlying disease or metabolic stress)
  • destructive myopathies
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8
Q

what 2 types of myopathies are characterized by having normal muscle mass?

A
  • channelopathies
  • metabolic myopathies
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9
Q

what are 3 types of channelopathies

A
  • Ca+ release
  • Cl- channel
  • Na+ channel
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10
Q

What are 2 types of metabolic myopathies

A
  • glycogenoses (McArdie and Tauri)
  • mitochondrial
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11
Q

what are 3 types of neuropathic muscle diseases

A
  • anterior horn cell (SMA)
  • peripheral neuropathies
  • neuromuscular junction (Myasthenia gravis)
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12
Q

what is an example of a common muscle missing at birth?
is the deficiency usually uni or bilateral?

A
  • palmaris longus
  • unilateral
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13
Q

what are 3 types of idiopathic autoimmune myopathies we focus on?

A

dermatomyositis
polymyositis
inclusion body myositis *

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14
Q

what are some general characteristics of idiopathic autoimmune myopathies?

A
  • typically myopathy in proximal muscles, usually symmetrically
  • weakness progresses faster than in muscular dystrophy, rarely occurs acutely
  • severe cases associated with muscle wasting
  • all treated with corticosteroids or immunosuppressants
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15
Q

which pathology is characterized by a rash, f>m, fine motor impairement, and perimysial inflammation

A

dermatomyositis

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16
Q

which myopathy occurs after the 2nd decade of life and affects proximal muscle before distal muscles

A

polymyositis

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17
Q

which pathology affects m>w, w>b, distal before proximal, 50’s, falls common due to quadricep weakness, and progresses slowly

A

inclusion body myositis

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18
Q

hyperkalemia?

A
  • elevated K level
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19
Q

hypokalemia

A
  • low K level
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20
Q

what is the cause of periodic paralysis

A

hyperkalemia and hypokalemia

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21
Q

what is the etiology of kyper and hypokalemia?

A
  • fluid loss, disease, meds, hereditary
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22
Q

onset of periodic paralysis is often due to?

A

exercise

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23
Q

what disease happens in type 1 muscle and is due to an issue with RYR1 releasing Ca?

A

core myopathy/central core disease

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24
Q

what is malignant hyperthermia causes by?

A

RYR mutation

  • body temp goes crazy under anesthetics and the fever can kill you
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25
Q

what is the opposite of core myopathy?

A

brody syndrome

26
Q

what is brody’s syndrome

A

progressive muscle stiffness and pain during exercise

27
Q

what disease happens in type 2 muscle and is an issue with SERCA pumping Ca+ back into the SR?

A

brody’s syndrome

28
Q

do malignant hyperthermia, hypo/hyperkalaemic paralysis and myotonia cause change in muscle mass?

A

no because they are all channelopathies

29
Q

what disease is characterized by an issue with Cl conductance and issues repolarizing the cell.

A

myotonia congenita

30
Q

what are two types of myotonia congenita

A
  • thomsen disease
  • becker disease
31
Q

is becker or thomsen disease more severe?

A

becker

32
Q

what do people with thomsen disease struggle with?

A
  • falls, grasping objects, chewing
  • muscle size and strength
33
Q

what do people with becker disease primarily struggle with?

A
  • gait issues and unprotected falls
34
Q

thomsen and becker diseases are due to _____ conductance problems

A

Cl-

35
Q

what is the most common myopathy?

A

alcoholic myopathy

36
Q

T or F: alcohol influences multiple aspects of both the anabolic and catabolic arms of the pathway

A

T

37
Q

T or F: cirrhosis of the liver is more common than myopathy in alcoholics

A

F, myopathy is more common

38
Q

what are the two forms of alcoholic myopathy?

A

chronic and acute

39
Q

is chronic or acute alcohol myopathy characterized by being painless, mild proximal weakness and affects pelvic more than shoulder girdle musculature

A

chronic

40
Q

is acute or chronic alcohol myopathy characterized pain, muscle swelling, false DVT positive, compartment syndrome, rhabdomyolysis and usually affects 1-2 muscles

A

acute

41
Q

what are some pathogenic mechanisms leading to alcohol-induced muscle damage

A
  • carbs, protein, and energy metabolism
  • oxidative balance
  • gene dysregulation
  • cell turnover
  • signal transduction
  • apoptosis
42
Q

increased alcohol abuse = ________ strength

A

decrease

43
Q

how does alcohol abuse affect muscle synthesis

A

decreases it

44
Q

does strength improve with abstinence and low-dose controlled consumption

A

yes

45
Q

does alcohol abstinence restore protein synthesis?

A

yes

46
Q

is there increased skeletal muscle apoptosis in alcoholics?

A

yes, TUNEL assay proves that

47
Q

is skeletal muscle apoptosis between chronic alcoholics with and without myopathy similar?

A

no those with myopathy have much larger rates of apoptosis

48
Q

how is the degree of alcoholic myopathy rates

A

mild, moderate, sever

49
Q

are men and women equally affected by alcoholic myopathy

A

no, men affected more than women

50
Q

what exacerbates alcoholic myopathy

A

critical illness
hospitalization
infections (HIV)
malnutrition
inactivity

51
Q

what mitigates alcoholic myopathy

A
  • exercise
  • myostatin inhibitors
  • good nutrition
52
Q

what are the 3 types of ehlers-danlos syndrome we need to remember

A

hypermobility
classical
vascular

53
Q

hypermobility EDS is characterized by:

A

extreme flexibility

54
Q

classical type EDS affects what type of collagen

A

V and 1

55
Q

skin hyperextensibility, widened atrophic scars, and joint hypermobility are characteristics of what type of EDS

A

classical type

56
Q

what type of collagen is affected in vascular EDS

A

3

57
Q

most serious form, arterial/organ/tendon/muscle rupture, digit joint hypermobility are characteristics of what type of EDS?

A

vascular

58
Q

what are two types of carb metabolism myopathy we are concerned with

A

McArdle disease
Tauri disease

59
Q

what is affected in McArdle disease? why is that bad?

A

myophosphorylase

body can’t break glycogen down into glucose in the muscles

60
Q

what is affected in Tarui disease and why is that bad?

A

phosphofructokinase (PFK) enzyme deficiency
that’s bad because this enzyme is needed in glycolysis. if we don’t have it your body can’t use glucose as fuel

61
Q

T or F: lipid storage diseases can happen anywhere

A

T