Neuromuscular Disorders Flashcards

1
Q

What is amyotrophic lateral sclerosis (ALS)?

A

Muscle weakness caused by degeneration of motor neurons

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2
Q

Describe the pathophysiology of ALS (3)

A
  • Begins with degeneration of LMNs (in spinal cord)
  • Followed by degeneration of UMNs (in brain)
  • As nerve cells die, muscles atrophy
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3
Q

Describe the progression of ALS

A

Upper extremities –> neck / throat –> trunk –> lower extremities

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4
Q

When are patients with ALS admitted? (2)

A
  • Diagnosis
  • Final stage of debilitation
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5
Q

The onset of death associated with ALS is within ______

A

5 years

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6
Q

Death from ALS is often due to ______

A

Pneumonia

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7
Q

What are the LMN manifestations of ALS? (3)

A
  • Atrophy
  • Fatigue
  • Muscle weakness
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8
Q

What are the UMN manifestations of ALS? (2)

A
  • Spastitity
  • Hyperreflexia
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9
Q

What are the most common manifestations of ALS? (2)

A
  • Dysphagia
  • Dysarthria
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10
Q

What is dysarthria?

A

Slurred speech

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11
Q

Describe the primary goal of ALS treatment

A

Supportive care - no cure

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12
Q

What factor is required for the diagnosis of ALS?

A

Presence of anterior horn dysfunction in 3 limbs

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13
Q

What is the primary medication used for the treatment of ALS?

A

Minocycline - inhibits motor neuron death

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14
Q

Describe the nursing interventions associated with ALS (4)

A
  • Assistive devices
  • Conserve energy
  • Prevent aspiration
  • Encourage small, frequent, high-nutrient meals
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15
Q

What functions remain intact for a patient with ALS? (3)

A
  • Cognition
  • Sensation
  • Bowel / bladder control
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16
Q

What are the priority assessments associated with ALS? (2)

A
  • Respiratory function
  • Swallowing function
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17
Q

What is myasthenia gravis (MG)?

A

Weakness / fatigue of voluntary muscles

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18
Q

Describe the pathophysiology of MG (3)

A
  • Antibodies act against acetylcholine receptors
  • Decreased acetylcholine
  • Increased cholinesterase
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19
Q

Acetylcholine is responsible for ______

A

Muscle contraction

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20
Q

Cholinesterase is responsible for ______

A

Breakdown of acetylcholine

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21
Q

What are the manifestations of MG? (4)

A
  • Asymmetrical facial drooping
  • Downward turn of the mouth
  • Ptosis
  • Diplopia
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22
Q

What is ptosis?

A

Eyelid drooping

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23
Q

What is diplopia?

A

Double vision

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24
Q

What functions are most affected by MG? (4)

A
  • Facial expressions
  • Chewing
  • Swallowing
  • Speech
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25
Q

What is the primary diagnostic test used for the diagnosis of MG?

A

Anticholinesterase testing - tensilon

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26
Q

Describe the MOA of tensilon

A

Increases acetylcholine at neuromuscular junctions by blocking cholinesterase

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27
Q

How is tensilon used for the diagnosis of MG? (2)

A
  • Improvement of muscle strength - indication of MG diagnosis
  • No improvement of muscle strength - rules out MG diagnosis
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28
Q

What medications are used for the treatment of MG? (3)

A
  • Prostigmin - improves muscle strength
  • Mestinon - improves muscle strength
  • IV Atropine - counteracts cholinergic reactions
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29
Q

When administering MG medications, it is important to monitor for ______

A

Cholinergic side effects

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30
Q

What are some examples of cholinergic side effects? (3)

A
  • Excessive salivation
  • Sweating
  • Diarrhea
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31
Q

Describe the primary nursing consideration associated with MG medications

A

Must be given at exact same time everyday

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32
Q

What are the complications associated with MG? (2)

A
  • Myasthenic crisis
  • Cholinergic crisis
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33
Q

Myasthenic crisis is caused by ______

A

Under-medication of anticholinesterase drugs

34
Q

Describe the treatment of a myasthenic crisis (2)

A
  • Increase anticholinesterase drugs
  • Intubation / mechanical ventilation
35
Q

If patient responds to mechanical ventilation within 48 hours …

A

Wean off of ventilator

36
Q

Cholinergic crisis is caused by ______

A

Over-medication of anticholinesterase drugs

37
Q

What paradoxical reaction occurs in the case of a cholinergic crisis?

A

Bronchospasm

38
Q

Describe the treatment of a cholinergic crisis

A

IV atropine - 1 mg

39
Q

Improvement after administration of tensilon indicates a ______ crisis

A

Myasthenic

40
Q

Worsening after administration of tensilon indicates a ______ crisis

A

Cholinergic

41
Q

Treatment of myasthenic crisis can often lead to ______

A

Cholinergic crisis

42
Q

Describe the nursing interventions associated with MG (3)

A
  • Encourage coughing / deep breathing
  • Eye protection / lubrication
  • Eye patching for diplopia
43
Q

What is multiple sclerosis (MS)?

A

Disruption of CNS pathways caused by myelin sheath damage

44
Q

Which disorder is characterized by remissions and exacerbations?

A

MS

45
Q

What are the manifestations of MS? (4)

A
  • Paresthesia
  • Optic neuritis
  • Spastic paralysis
  • Bowel / bladder dysfunction
46
Q

______ is the first / earliest manifestation of MS

A

Vision loss

47
Q

What finding requires immediate action upon assessment of a patient with MS?

A

Dysphagia - risk of aspiration

48
Q

What is the primary diagnostic test used in the diagnosis of MS?

A

Lumbar puncture - elevated WBCs / proteins

49
Q

What medications are used for the treatment of MS? (4)

A
  • Anticholinergics
  • Corticosteroids
  • Imuran / cytoxan
  • Interferon (antiviral)
50
Q

What is Guillain Barre syndrome?

A

Peripheral nerve damage caused by infection / virus

51
Q

Guillain Barre syndrome is preceded by ______ 1 - 4 weeks prior to onset

A

Flu-like symptoms

52
Q

Manifestations of Guillain Barre syndrome occur …

A

In an ascending motor fashion

53
Q

Manifestations of Guillain Barre syndrome recover …

A

In a descending motor fashion

54
Q

What are the manifestations of Guillain Barre syndrome? (4)

A
  • Paresthesia
  • Vocal paralysis
  • Respiratory paralysis
  • Orthostatic hypotension
55
Q

What functions remain intact for a patient with Guillain Barre syndrome? (2)

A
  • Consciousness
  • Cerebral function
56
Q

What is the primary diagnostic test used for the diagnosis of Guillain Barre syndrome?

A

Lumbar puncture - elevated WBCs / proteins

57
Q

Describe the primary goal of Guillain Barre syndrome treatment

A

Supportive care - no cure

58
Q

What is the primary medication used for the treatment of Guillain Barre syndrome?

A

IV immunoglobulin (IVIG)

59
Q

When administering IV immunoglobulin, it is important to ensure that the patient is ______

A

Well hydrated

60
Q

What is the primary procedure used for treatment of Guillain Barre syndrome?

A

Plasmapheresis - removal of antibodies from plasma

61
Q

Plasmapheresis must be used within the first ______ of Guillain Barre syndrome

A

2 weeks

62
Q

What is trigeminal neuralgia (tic douloureux)?

A

Irritation of CN V - ophthalmic / maxillary / mandibular

63
Q

What are the manifestations of trigeminal neuralgia? (2)

A
  • Twitching of facial muscles (tic douloureux)
  • Sudden pain - caused by trigger
64
Q

Pain from trigeminal neuralgia is ______ by analgesics

A

Unrelieved

65
Q

Trigeminal neuralgia causes difficulty ______

A

Chewing

66
Q

What are the possible triggers associated with trigeminal neuralgia pain? (3)

A
  • Touching the face
  • Heat / cold
  • Talking
67
Q

What medications are used for the treatment of trigeminal neuralgia? (2)

A
  • Tegretol
  • Baclofen
68
Q

What is Bell’s Palsy?

A

Unilateral facial paralysis due to damage to CN VII

69
Q

What are the manifestations of Bell’s Palsy? (3)

A
  • Inability to raise eyebrow / smile on affected side
  • Flattened nasolabial fold
  • Bell’s phenomenon
70
Q

Describe Bell’s phenomenon

A

Upward rolling of the eyeball when blinking

71
Q

Describe the primary nursing intervention associated with Bell’s Palsy

A

Massage the face in upward motion TID - prevents atrophy

72
Q

What are the complications associated with Bell’s Palsy? (2)

A
  • Corneal ulcerations
  • Blindness
73
Q

What is Lyme disease?

A

Inflammatory disease caused by ticks

74
Q

The chances of developing Lyme disease are dependent on what 3 factors?

A
  • The type of tick
  • Where the patient was when the bite occurred
  • How long the tick remained attached
75
Q

What are the manifestations of Lyme disease? (4)

A
  • Rash
  • Fever
  • Joint pain
  • Swollen lymph nodes
76
Q

Manifestations of Lyme disease occur ______ after a tick bite

A

3 - 30 days

77
Q

Neurological deficits occur during what stages of Lyme disease?

A

2 - 3

78
Q

What neurological deficits are common during stage 2 of Lyme disease? (2)

A
  • Peripheral neuropathy
  • Bell’s Palsy
79
Q

What is the primary diagnostic test used in the diagnosis of Lyme disease?

A

2-step blood testing for antibodies

80
Q

Blood testing for diagnosis of Lyme disease must take place …

A

After symptoms are present

81
Q

What medications are used for the treatment of Lyme disease?

A

IV antibiotics - amoxicillin / tetracycline