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NUNA 684 Advanced Pathophysiology > Exam IV: Myasthenia Gravis > Flashcards

Exam IV: Myasthenia Gravis Flashcards

1
Q

Chronic disease of _____ _____ (____)
Most _____ neuromuscular disorder

A

neuromuscular junction (nmj)
prevalent

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2
Q

Ranges ___-____ per 1,000,000 people

A

3-30

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3
Q

If <50, more _____ than ____ (3:2)
Common in ____

A

women than men
elderly

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4
Q

Manifested by: (3)

A
  • Increasing skeletal muscle weakness
  • Fatigue of muscles with effort
  • Partial restoration of strength/function with rest
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5
Q

Decreased # of working ACh receptors _______; ________ ACh pool is normal

A

postsynaptically
prejunctional

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6
Q

_____ found in 80-85% of MG patients

A

Antibodies

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7
Q

Unknown promotion production of ___ antibodies—_____ gland seems to play central role
*______ improves symptoms, not curative

A

IgE
Thymus
Thymectomy

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8
Q

Only a _____ disease - intact sensation, ANS, and cognition

A

MOTOR

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9
Q

Increased prevalence in those living close to the ______

A

equator

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10
Q

**Decreased # of working ACh receptors _______; ________ ACh pool is all normal.

A

postsynaptically
Pre-junctional

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11
Q

Hallmark: _____ _____ that improves with rest; inability to sustain/repeat _____ _____

A

Generalized weakness
muscular contractions

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12
Q

more movement =

A

more weakness

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13
Q

Wide range of symptoms — slight ptosis —–> ____ ____

A

respiratory failure

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14
Q

Eyes, mouth, pharynx, proximal limb, and shoulder girdle muscles =

A

most often affected

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15
Q

MG exacerbated in 33% of pregnant women with MG, can produce ______________________ (less than 20%)

A

transitory symptoms in newborns

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16
Q

Exacerbated by:

A

surgery, electrolyte imbalances, some medications

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17
Q

treatment: ______ inhibitors - (4)

A

*Pyridostigmine
*(60mg, tid= 2mg IV)
*Immunosuppressants
*Thymectomy
*Plasmapheresis

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18
Q

CHOLINERGIC CRISIS
overdosing of anticholinesterase
Sx: ______ stimulation - excessive ____, _____, _____, ______, _____. Weakness and ____ _____

A

Muscarinic stimulation: excessive salivation, diarrhea, excessive tearing, bradycardia, miosis
respiratory failure

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19
Q

MYASTHENIC CRISIS
_______ of anticholinesterase
Sx: pupils ______, ______, etc. Weakness and _____ _____.

A

Underdosing
Pupils normal size, ptosis, etc.
Weakness and respiratory failure

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20
Q

Anesthesia Implications

Evaluate–disease controlled? anticholinesterase dose stable? –> ___ ____ and ____ ____ surgery

A

sev days
right before

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21
Q

if meds taken DOS - (3)

A

increased vagal reflex
interferes with muscle relaxants
inhibits plasma cholinesterase

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22
Q

if dose withheld and if disease is advanced/possible deterioration (2)

A

have aspiration risk
at risk for respiratory failure

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23
Q

Review electrolytes and correct if needed
Hypokalemia can _____ _____ _____

A

potentiate muscle weakness

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24
Q

When developing plan of care, must consider:

A

Pharyngeal/laryngeal muscle weakness
Oral secretions—difficulty eliminating
Increased risk of pulmonary aspiration

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25
Q

_____ and _____ muscle dysfunction accounts for much of morbidity of MG

A

Swallowing and respiratory

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26
Q

_____ or _____ anesthesia preferred

A

Regional or local

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27
Q

If GA — _____ _____ may decrease muscle tone enough for intubation; may not need muscle relaxants

A

inhalational agents

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28
Q

Sensitive to ______ NMBA (roc, vec, etc)

A

nondepolarizing

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29
Q

Reverse MR cautiously to avoid “____ ____”; sugammadex ___ -____ mg/kg if available

A

cholinergic crisis
2-4

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30
Q

Assess NM blockade at _____ _____ muscle; may overestimate degree of relaxation, but best place to avoid undetected residual weakness

A

orbicularis oculi

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31
Q

Post Op-EVALUATE FULLY!!!
Muscle strength seems adequate in _____ stages of recovery then can deteriorate ___________ later

A

early
a few hours

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32
Q

Predictors that post op ventilation may be needed:

A

transsternal thymectomy
having disease > 6 years
daily pyridostigmine dose > 750 mg
COPD
preop VC <2.9L

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33
Q

Succinylcholine (Sch)

If patient not treated, then ____ to Sch (maybe even 2-3x more _____)
RSI-dose is ___-___ mg/kg vs 1-1.5 mg/kg

A

resistant
resistant
1.5-2

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34
Q

Succinylcholine (Sch)

If patient on cholinesterase inhibitors, effects of Sch (and _____ local anesthetics) may be _____ (no hydrolysis/breakdown and it lingers)

A

ester
prolonged

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35
Q

Non-depolarizers (NDMR)
_____ dose or ____
(could be ___-___x more sensitive)

A

Decrease
avoid
10-100

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36
Q

NDMR - If needed, the use of ____ doses
(____-____ the dose of normal) of shorter acting NDMR is a wiser choice

A

small
1⁄2-2⁄3

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37
Q

NDMR - Better to titrate dose to allow for _____ _____ than to have to reverse

A

spontaneous recovery

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38
Q

CAUTION
May produce worsening myasthenic weakness:

A
  • Quinine, quinidine, and procainamide
  • Antibiotics: aminoglycosides(gent,neomycin,etc) quinolones (ciprofloxacin,levofloxacin,etc), and macrolides (erythromycin, azithromycin)
  • Beta blockers-systemic and ocular
  • Calcium channel blockers
  • Mag salts (including laxatives with high Mg2+)
  • Iodinated contrast
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39
Q

Anticholinesterases - ?impair hydrolysis of ester LA causing prolonged block; _____ _____ of LA appropriate (Lidocaine, Bupiv, Ropiv)

A

amide class

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40
Q

Lambert-EatonMyasthenic Syndrome (LEMS)

____/____ disorder
Usually occurs in those with _____ -especially those with small cell Ca of bronchi

A

Rare/autoimmune
malignancy

41
Q

Lambert-EatonMyasthenic Syndrome (LEMS)

___-___ of people show no evidence of Ca
Most patients = ____ b/t 50 and 70

A

1/3-1/2
men

42
Q

Lambert-EatonMyasthenic Syndrome (LEMS)

Seems that the _____ _____ channels are messed up via autoantibodies

A

presynaptic Ca++

43
Q

Lambert-EatonMyasthenic Syndrome (LEMS)

Reduction in Ca++ mediated exocytosis of ____ at _____

A

ACh at nmj

44
Q

Lambert-EatonMyasthenic Syndrome (LEMS)

Post-junctional AChRs are ______ - unlike _____

A

unaffected
MG

45
Q

Lambert-EatonMyasthenic Syndrome (LEMS)

Similar to that of ______ toxicity or _____ poisoning

A

Mg++
botulism

46
Q

Clinical Manifestations - LEMS
main features:

A
  • muscle weakness
  • fatigue
  • hyporeflexia
  • proximal limb muscle aches
47
Q

Clinical Manifestations - LEMS

____ and ____ muscles involved

A

Diaphragm and resp

48
Q

Clinical Manifestations - LEMS

Autonomic nervous system dysfunction leads ______ hypotension, impaired gastric _____, _____ retention

A

orthostatic
motility
urinary

49
Q

Clinical Manifestations - LEMS

Brief _____ in strength with voluntary contraction

A

INCREASE

50
Q

Clinical Manifestations - LEMS

_____ leads to progressive strengthening of muscle

A

Tetany

51
Q

LEMS - treatment

No _____

A

cure

52
Q

LEMS - treatment

goal is to improve _____ _____, decrease _____ deficits

A

muscle strength
ANS

53
Q

LEMS - treatment

__________ — helps some people b/c improves Ca+ influx, thus increasing amt of ACh released

A

3, 4-Diaminopyridine

54
Q

LEMS - treatment

_______, _______, IV ________, immunosuppressants help some patients

A

Plasmapheresis, corticosteroids
immunoglobulin

55
Q

LEMS - Anesthesia Implications

Must be suspected in patients with hx of _____ ______ and suspected dx of ____ of the lung

A

muscle weakness
cancer

56
Q

LEMS - Anesthesia Implications

VERY sensitive to both ______ and ______

A

depolarizing and NDMR

57
Q

LEMS - Anesthesia Implications

May use just inhalational agents alone for _____ _____

A

tracheal relaxation

58
Q

LEMS - Anesthesia Implications

Can use ______ to reverse

A

anticholinesterase

59
Q

LEMS - Anesthesia Implications

Possible to require post op ______/______, must discuss PREOP!

A

intubation/ventilation

60
Q

Myotonic Dystrophy

All the “myotonias” have the inability of skeletal muscles to _____ after ______ (chemical or physical)

A

relax after stimulation

61
Q

Myotonic Dystrophy

Also called “_____ _____”—is multisystem disease

A

Steinert’s disease

62
Q

Myotonic Dystrophy

Skeletal muscles are hypoplastic, dystrophic, and weak — yet ______ _____

A

persistently contract

63
Q

Myotonic Dystrophy

Usually occurs ___-___ years old

A

20-30

64
Q

Myotonic Dystrophy

Slow, progressive deterioration of ____ _____ ____

A

all muscle groups

65
Q

Patho and Tx-Myotonic Dystrophy

Myotonia = persistent _____ of muscle after ______; early in disease

A

contracture
stimulation

66
Q

Patho and Tx-Myotonic Dystrophy

Made worse by ____ and _____, warming room temp is helpful

A

cold and shivering

67
Q

Patho and Tx-Myotonic Dystrophy

_____, ______, ______ are used to treat myotonic contractures, They delay return of membrane excitation — blocks ____ _____

A

Phenytoin, procainamide, tocainide
Na+ influx

68
Q

Patho and Tx-Myotonic Dystrophy

____ do NOT HELP

A

NDMR

69
Q

Patho and Tx-Myotonic Dystrophy

____ _____ available for the muscle weakness

A

NO treatment

70
Q

MD clinical manifestations:

A
  • Hypoplastic, weak skeletal muscles of face and neck
  • Hypersomnolent; Sleep apnea
71
Q

MD clinical manifestations: Cardiac

A
  • 50-90% have conduction defects
  • 1st degree AV block is most common
  • Also see bradycardia, atrial flutter/fib
72
Q

MD clinical manifestations: Respiratory

A
  • Pharyngeal and resp muscle weakness
  • Restrictive type impairment
73
Q

MD clinical manifestations: Gastrointestinal

A

Intestinal hypomotility; gastric atony

74
Q

MD Anesthetic Implications:

Abnormal _____ makes them vulnerable to pulm _____

A

swallowing
aspiration

75
Q

MD Anesthetic Implications:

AVOID

A

hypothermia/shivering

76
Q

MD Anesthetic Implications:

preop ____ and close intraop monitoring

A

EKG

77
Q

MD Anesthetic Implications:

_____ — good choice; doesn’t prevent contractures

A

RA

78
Q

MD Anesthetic Implications:

Avoid succinylcholine; NDMR are ok but recognize/remember ____ ____

A

muscle wasting

79
Q

MD Anesthetic Implications:

be gentle with ____ and careful _____

A

airway
positioning

80
Q

Duchenne’s Muscular Dystrophy

Progressive, ______ degeneration and ______ of muscle fibers

A

painless
necrosis

81
Q

Duchenne’s Muscular Dystrophy

Pelvic girdle and muscle of thighs affected _____

A

1st

calf is where its seen first but these are where you notice that something is truly wrong

82
Q

Duchenne’s Muscular Dystrophy

Mutant gene identified on “X” chromosome in 1987; Error in coding “_____” protein
Duchenne’s – _____ ____
(Becker’s - ______ levels)

A

dystrophin
no dystrophin
decreased

83
Q

Duchenne’s Muscular Dystrophy

X-linked ______ — occurs in _____

A

recessive
males

84
Q

Duchenne’s Muscular Dystrophy

1 in _____ male births
Most _____ and most _____ of dystrophies

A

3500
common
severe

85
Q

DMD-Pathophysiology

Painless and progressive degeneration due to ______ of _____ and ______ tissue in the muscle
*first seen in ____ muscle

A

infiltration of fat and fibrous
calf

86
Q

DMD-Pathophysiology

Skeletal muscle-degeneration of _____ _____

A

respiratory muscles

87
Q

DMD-Pathophysiology

Cardiac muscle - cardiomyopathy, mitral _____, _____ dysrhythmias
EKG changes - tall R waves (rt) and deep Q waves (left)
Limited activity ____ _____ underlying ____-____ condition

A

regurg
ventric
may mask
cardio-pulm

88
Q

DMD-Pathophysiology

smooth muscle -

A

GI

89
Q

DMD - ______ sign

A

Gower’s

90
Q

Gower’s Sign:
Presents _________
Wheelchair by ________
Death _______

A
  • presents early - 2 to 6 year old
  • wheelchair by 12
  • death usually in late teens, early adulthood (pneumonia or CHF)
91
Q

DMD AIs:

_____/_____ anesthesia = ideal; TIVA

A

Local/regional

92
Q

DMD AIs:

chronic _____ — be sure to cover during surgery

A

steroids

93
Q

DMD AIs:

Opioids, sedatives, GA, patient very sensitive; use ____ _____ possible

A

smallest amount

94
Q

DMD AIs:

Careful titration/monitoring with NDMR - _____ effect (___-___ ____)

A

prolonged
3-6x longer

95
Q

DMD AIs:

_______ is contraindicated!

A

Succinylcholine (think MH type symptoms)

96
Q

DMD AIs:

Carefully titrate fluids d/t risk of _____

A

CHF (micro drip)

97
Q

DMD AIs:

______ + ______ = heart failure

A

Hypotension + tachycardia

98
Q

DMD AIs:

aspiration risk - consider _____

A

NGT

NUNA 684 Advanced Pathophysiology (16 decks)

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