1: Neuromuscular & Rare (Part 1) Flashcards

1
Q

where is smooth/involuntary muscle found

A

within the walls of organs and structures such as the esophagus, stomach, intestines, bronchi, uterus, urethra, bladder, blood vessels, and the arrector pili in the skin.

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2
Q

what muscle is under conscious control

A

skeletal

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3
Q

what 2 muscles are striated, in that they contain sarcomeres and are packed into highly regular, repeating arrangements of bundles

A

cardiac & skeletal muscles

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4
Q

____________ muscles are arranged in regular, parallel bundles

A

skeletal

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5
Q

____________ muscle connects at branching, irregular angles, calledintercalated discs.

A

cardiac

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6
Q

Striated muscle contracts and relaxes in ____________ bursts

A

short, intense

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7
Q

is cardiac muscle more similar to smooth or skeletal muscle

A

skeletal

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8
Q

what part of the NMJ contains acetylcholinesterase

A

synaptic cleft

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9
Q

what part of the NMJ releases acetylcholine into the cleft

A

presynaptic storage vesicles

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10
Q

acetylcholine diffuses across the synaptic cleft and binds to the

A

nicotinic cholinergic receptor

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11
Q

what happens when depolarization reaches the nerve terminal

A

voltage gated Ca++ channels open

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12
Q

what subunits are capable of binding acetylcholine

A

only the 2 α- subunits

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13
Q

what happens during influx of calcium

A

Ach vesicles fuse with the nerve plasma membrane and then

Expel their content into the synaptic cleft

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14
Q

the amount of acetylcholine released is influenced by

A

the amount of calcium that enters the nerve terminal during nerve stimulation.

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15
Q

what channels are affected by aminoglycoside abx

A

inhibit Calcium channels and Ach release at the NMJ

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16
Q

upregulation

A

When frequency of stimulation of NMJ decreases over days due to severe burns, immobilization, infection, sepsis, prolonged use of NMBAs in ICU, CVAs

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17
Q

do the # of immature nAChRs increase or decrease in upregulation

A

increase

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18
Q

in upregulation, immature nAChRs will have increased sensitivity to

A

acetylcholine and succinylcholine

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19
Q

in upregulation, immature nAChRs will have decreased sensitivity to

A

non depolarizers

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20
Q

downregulation can be caused by

A

chronic neostigmine use (too much acetylcholine around)

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21
Q

multiple sclerosis patho

A

immune-mediated inflammatory demyelinating disease of the CNS

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22
Q

treatment for multiple sclerosis

A

corticosteroids
β interferons
glatiramer acetate

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23
Q

main symptoms of multiple sclerosis (picture)

A
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24
Q

MS is a demyelinating disease of

A

the brain and spinal cord

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25
Q

multiple sclerosis is mediated by

A

T-cell autoantibodies against myelin

converts T-cells into inflammatory cells

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26
Q

are peripheral nerves affected with MS

A

NO

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27
Q

who is more likely to have MS males or females

A

females x8

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28
Q

when do symptoms generally appear with MS

A

between 20-40 yrs of age

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29
Q

diagnosis of MS

A

2 or more attacks separated by a month or more

Involvement of two or more noncontiguous anatomic areas

Elevated levels of IgG and albumin in the CSF

MRI: demyelinated plaques in the CNS

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30
Q

do MS exacerbations ↑ or ↓ in pregnancy

A

decrease

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31
Q

MS during the postpartum period

A

may have an increased risk of relapse

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32
Q

goal of treatment for MS

A

modulate the immunologic and inflammatory responses that damage the CNS

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33
Q

common side effects of interferon β

A

flu like symptoms, elevated liver enzymes and neutropenia are common side effects

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34
Q

2 types of meds that may be used in treatment of MS

A

disease-modifying agents (interferon β )

immunosuppressants (azathioprine), corticosteroids, IV immunoglobulins

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35
Q

what are some symptomatic treatments for MS

A

gabapentin, baclofen and SSRIs may be treatments for spasticity, depression and neuropathic pain

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36
Q

what is Lhermitte sign

A

Neck flexion induces an “electrical sensation”

seen its patients with MS

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37
Q

what in Unthoff sign

A

Worsening symptoms by increased body temperature (exercise, hot temps) seen in patients with MS

38
Q

anesthetic implications of MS

A

Exacerbation of symptoms with hyperthermia, stress, surgery, infection, emotional and physical trauma, postpartum

39
Q

spinal anesthesia and MS

A

Spinal anesthesia may exacerbate symptoms due to potential neurotoxicity (questionable)

Epidural and peripheral blocks okay

40
Q

what NMBA should be avoided in patients with MS

A

succinylcholine

41
Q

alzheimer’s protein is associated with what protein

A

amyloid-β protein

42
Q

amyloid-β protein begins of a cascade of events ending with deposits of (3)

A
  • amyloid plaques
  • neurofibrillary tangles
  • neuronal apoptosis
43
Q

alzheimer’s consists of a loss of ____________ activity

A

cholinergic

44
Q

Alzheimer Disease is characterized by

A

These cause a loss of cholinergic activity and loss of glutamatergic neurons

45
Q

treatment of Alzheimer Disease consists of

A

acetylcholinesterase inhibitors and a NMDA inhibitor

46
Q

anesthesia considerations for patients with Alzheimer Disease (3)

A
  • Postoperative cognitive dysfunction, careful with sedatives premeds
  • Use glycopyrrolate
  • Patients already receiving cholinesterase inhibitors may have prolonged Sux response
47
Q

parkinson disease patho

A

Degenerative CNS disease caused by loss of dopaminergic cells in the basal ganglia (Lewy Bodies)

48
Q

Parkinson clinical manifestations

A

Resting tremor, cogwheel rigidity of the upper extremities, bradykinesia, shuffling gait, stooped posture, and facial immobility (frozen face)

49
Q

what is diminished during Parkinson Disease

A

Diminished inhibition of the extrapyramidal motor system due to dopamine deficiency

50
Q

treatment of Parkinson disease (2)

A

Levodopa (multiple other drugs available) and Deep-brain stimulators

51
Q

half life of Levodopa

A

Half life of Levodopa is short, interruption of therapy for more than 6-12 hours can result in severe skeletal muscle rigidity and ventilation issues

52
Q

what meds to avoid in Parkinson disease

A

Avoid dopamine antagonists (Reglan, Phenergan)

Increased risk of neuroleptic Syndrome with dopamine agonists

53
Q

what meds are dopamine antagonists

A

as phenothiazines, droperidol, and metoclopramide

54
Q

alfentanil and fentanyl with parkinson disease

A

fentanyl may produce dystonic reactions when administered rapidly.

55
Q

deep brain stimulator (DBS) surgery

A

Two-part surgery: Stimulator and generator placement

56
Q

DBS is most effective in people who suffer from

A

severe tremors

57
Q

effects of DBS surgery

A

Suffer wearing-off spells or medication induces dyskinesias (uncontrolled shakes)

Does not slow disease process

58
Q

motor neuron disorders

A

Upper, lower or mixed motor neuron involvement of the cerebral cortex, brainstem and spinal cord

59
Q

what motor neurons are affected in ALS

A

mixed

60
Q

what motor neurons are affected in Kennedy’s disease

A

mixed

61
Q

what motor neurons are affected in spinal muscular atrophy

A

lower motor neurons

62
Q

who is more affected from ALS men or women

A

males, onset 40-60 yrs

63
Q

diagnosis of ALS

A

EMG and neuro exam
early spastic weakness of the upper and lower extremities,
subcutaneous muscle fasciculations,
bulbar involvement affecting pharyngeal function, speech and facial muscles

64
Q

ALS patho

A

Progressive degeneration of the upper and lower motor neurons causing:

amyotrophy (muscle wasting)

lateral sclerosis

65
Q

treatment of ALS

A

none curative, treat symptomatically

66
Q

bulbar involvement and ALS

A

aspiration risk and pulm complications

67
Q

patients with ALS have ↑ sensitivity to

A

respiratory depressant effects of sedatives and hypnotics (avoid opioids and benzos)

68
Q

what NMBA to avoid in patients with ALS

A

Avoid succinylcholine (risk of hyperkalemia due to denervation and immobilization)

69
Q

nondepolarizing agents in patients with ALS

A

may be prolonged and pronounced

70
Q

hyperkalemic periodic paralysis

A

Sodium channel defect cause prolonged depolarization & flaccid paralysis

Potassium >5.5 mEq/L during symptoms

71
Q

precipitating factors with hyperkalemic periodic paralysis

A

Rest after exercise 
Potassium infusions 
Metabolic acidosis 
Cold exposure (OR Hypothermia)

72
Q

in which periodic paralysis may skeletal muscle weakness may be localized to tongue and eyelids

A

hyperkalemic

73
Q

hypokalemic periodic paralysis

A

Calcium or Sodium channel defect

Potassium level <3 mEq/L during symptoms

74
Q

precipitating factors of hypokalemic periodic paralysis

A

High glucose meals 
Strenuous exercise 
Glucose-insulin infusions 
Stress 
Hypothermia

75
Q

chronic myopathy with aging is associated with

A

hypokalemic periodic paralysis

76
Q

periodic paralysis anesthesia management

A
77
Q

Guillain-Barre Syndrome general description (picture)

A
78
Q

Guillain-Barré syndrome is what kind of disorder

A

autoimmune

79
Q

polyradiculoneuritis is also called

A

Guillain-Barré syndrome

80
Q

Guillain-Barré syndrome clinical manifestations

A

Autonomic dysfunction with wide fluctuations in HR and BP

Physical stimulation can precipitate HTN, Tachycardia, and cardiac dysrhythmias

Respiratory muscle weakness

81
Q

treatment of Guillain-Barré syndrome

A

based on symptoms, plasma exchange, immunoglobulin, mechanical ventilation

82
Q

anesthetic considerations for patients with Guillain-Barré syndrome

A

Avoid rapid movement of patient (autonomics)

Aspiration (reglan,pepcid)

Maintain temperature

Airway Respiratory failure

Maintain preload and afterload

Careful with PPV (may cause autonomic instability)

Muscle relaxants: (AVOID Sux), increased sensitivity to NDMR

83
Q

myasthenia gravis is caused by…

A

a decrease in the number of functional postsynaptic, acetylcholine receptors in the neuromuscular junction available for acetylcholine binding

84
Q

MG patho

A

Autoimmune neuromuscular disease characterized by a decrease in acetylcholine receptors secondary to their destruction or inactivation by circulating IgG antibodies at post-synaptic nicotinic receptors in skeletal muscle

85
Q

abnormal ____________ tissue is frequently involved in patients with MG

A

thymus

86
Q

hallmark sign of MG

A

Fatigability which improves with rest

87
Q

major antigen in MG is the ____________ receptor

A

acetylcholine receptor (AChR) (on the muscle membrane)

88
Q

Some patients without AChR antibodies have autoantibodies against the

A

Muscle Specific Kinase (MuSK)

89
Q

Protein that allows AChR clustering at the NMJ

A

MuSK

90
Q

Receptor for neural agrin that relays the signal to MuSK to initiate AChR clustering

A

LRP4

91
Q

Patients without detectable antibodies against any of these three antigens are referred to as

A

seronegative