Neuromuscular Disorders Flashcards
1
Q
What is amyotrophic lateral sclerosis (ALS)?
A
Muscle weakness caused by degeneration of motor neurons
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
3
Q
Describe the progression of ALS
A
Upper extremities –> neck / throat –> trunk –> lower extremities
4
Q
When are patients with ALS admitted? (2)
A
- Diagnosis
- Final stage of debilitation
5
Q
The onset of death associated with ALS is within ______
A
5 years
6
Q
Death from ALS is often due to ______
A
Pneumonia
7
Q
What are the LMN manifestations of ALS? (3)
A
- Atrophy
- Fatigue
- Muscle weakness
8
Q
What are the UMN manifestations of ALS? (2)
A
- Spastitity
- Hyperreflexia
9
Q
What are the most common manifestations of ALS? (2)
A
- Dysphagia
- Dysarthria
10
Q
What is dysarthria?
A
Slurred speech
11
Q
Describe the primary goal of ALS treatment
A
Supportive care - no cure
12
Q
What factor is required for the diagnosis of ALS?
A
Presence of anterior horn dysfunction in 3 limbs
13
Q
What is the primary medication used for the treatment of ALS?
A
Minocycline - inhibits motor neuron death
14
Q
Describe the nursing interventions associated with ALS (4)
A
- Assistive devices
- Conserve energy
- Prevent aspiration
- Encourage small, frequent, high-nutrient meals
15
Q
What functions remain intact for a patient with ALS? (3)
A
- Cognition
- Sensation
- Bowel / bladder control
16
Q
What are the priority assessments associated with ALS? (2)
A
- Respiratory function
- Swallowing function
17
Q
What is myasthenia gravis (MG)?
A
Weakness / fatigue of voluntary muscles
18
Q
Describe the pathophysiology of MG (3)
A
- Antibodies act against acetylcholine receptors
- Decreased acetylcholine
- Increased cholinesterase
19
Q
Acetylcholine is responsible for ______
A
Muscle contraction
20
Q
Cholinesterase is responsible for ______
A
Breakdown of acetylcholine
21
Q
What are the manifestations of MG? (4)
A
- Asymmetrical facial drooping
- Downward turn of the mouth
- Ptosis
- Diplopia
22
Q
What is ptosis?
A
Eyelid drooping
23
Q
What is diplopia?
A
Double vision
24
Q
What functions are most affected by MG? (4)
A
- Facial expressions
- Chewing
- Swallowing
- Speech
25
What is the primary diagnostic test used for the diagnosis of MG?
Anticholinesterase testing - tensilon
26
Describe the MOA of tensilon
Increases acetylcholine at neuromuscular junctions by blocking cholinesterase
27
How is tensilon used for the diagnosis of MG? (2)
- Improvement of muscle strength - indication of MG diagnosis
- No improvement of muscle strength - rules out MG diagnosis
28
What medications are used for the treatment of MG? (3)
- Prostigmin - improves muscle strength
- Mestinon - improves muscle strength
- IV Atropine - counteracts cholinergic reactions
29
When administering MG medications, it is important to monitor for ______
Cholinergic side effects
30
What are some examples of cholinergic side effects? (3)
- Excessive salivation
- Sweating
- Diarrhea
31
Describe the primary nursing consideration associated with MG medications
Must be given at exact same time everyday
32
What are the complications associated with MG? (2)
- Myasthenic crisis
- Cholinergic crisis
33
Myasthenic crisis is caused by ______
Under-medication of anticholinesterase drugs
34
Describe the treatment of a myasthenic crisis (2)
- Increase anticholinesterase drugs
- Intubation / mechanical ventilation
35
If patient responds to mechanical ventilation within 48 hours ...
Wean off of ventilator
36
Cholinergic crisis is caused by ______
Over-medication of anticholinesterase drugs
37
What paradoxical reaction occurs in the case of a cholinergic crisis?
Bronchospasm
38
Describe the treatment of a cholinergic crisis
IV atropine - 1 mg
39
Improvement after administration of tensilon indicates a ______ crisis
Myasthenic
40
Worsening after administration of tensilon indicates a ______ crisis
Cholinergic
41
Treatment of myasthenic crisis can often lead to ______
Cholinergic crisis
42
Describe the nursing interventions associated with MG (3)
- Encourage coughing / deep breathing
- Eye protection / lubrication
- Eye patching for diplopia
43
What is multiple sclerosis (MS)?
Disruption of CNS pathways caused by myelin sheath damage
44
Which disorder is characterized by remissions and exacerbations?
MS
45
What are the manifestations of MS? (4)
- Paresthesia
- Optic neuritis
- Spastic paralysis
- Bowel / bladder dysfunction
46
______ is the first / earliest manifestation of MS
Vision loss
47
What finding requires immediate action upon assessment of a patient with MS?
Dysphagia - risk of aspiration
48
What is the primary diagnostic test used in the diagnosis of MS?
Lumbar puncture - elevated WBCs / proteins
49
What medications are used for the treatment of MS? (4)
- Anticholinergics
- Corticosteroids
- Imuran / cytoxan
- Interferon (antiviral)
50
What is Guillain Barre syndrome?
Peripheral nerve damage caused by infection / virus
51
Guillain Barre syndrome is preceded by ______ 1 - 4 weeks prior to onset
Flu-like symptoms
52
Manifestations of Guillain Barre syndrome occur ...
In an ascending motor fashion
53
Manifestations of Guillain Barre syndrome recover ...
In a descending motor fashion
54
What are the manifestations of Guillain Barre syndrome? (4)
- Paresthesia
- Vocal paralysis
- Respiratory paralysis
- Orthostatic hypotension
55
What functions remain intact for a patient with Guillain Barre syndrome? (2)
- Consciousness
- Cerebral function
56
What is the primary diagnostic test used for the diagnosis of Guillain Barre syndrome?
Lumbar puncture - elevated WBCs / proteins
57
Describe the primary goal of Guillain Barre syndrome treatment
Supportive care - no cure
58
What is the primary medication used for the treatment of Guillain Barre syndrome?
IV immunoglobulin (IVIG)
59
When administering IV immunoglobulin, it is important to ensure that the patient is ______
Well hydrated
60
What is the primary procedure used for treatment of Guillain Barre syndrome?
Plasmapheresis - removal of antibodies from plasma
61
Plasmapheresis must be used within the first ______ of Guillain Barre syndrome
2 weeks
62
What is trigeminal neuralgia (tic douloureux)?
Irritation of CN V - ophthalmic / maxillary / mandibular
63
What are the manifestations of trigeminal neuralgia? (2)
- Twitching of facial muscles (tic douloureux)
- Sudden pain - caused by trigger
64
Pain from trigeminal neuralgia is ______ by analgesics
Unrelieved
65
Trigeminal neuralgia causes difficulty ______
Chewing
66
What are the possible triggers associated with trigeminal neuralgia pain? (3)
- Touching the face
- Heat / cold
- Talking
67
What medications are used for the treatment of trigeminal neuralgia? (2)
- Tegretol
- Baclofen
68
What is Bell's Palsy?
Unilateral facial paralysis due to damage to CN VII
69
What are the manifestations of Bell's Palsy? (3)
- Inability to raise eyebrow / smile on affected side
- Flattened nasolabial fold
- Bell's phenomenon
70
Describe Bell's phenomenon
Upward rolling of the eyeball when blinking
71
Describe the primary nursing intervention associated with Bell's Palsy
Massage the face in upward motion TID - prevents atrophy
72
What are the complications associated with Bell's Palsy? (2)
- Corneal ulcerations
- Blindness
73
What is Lyme disease?
Inflammatory disease caused by ticks
74
The chances of developing Lyme disease are dependent on what 3 factors?
- The type of tick
- Where the patient was when the bite occurred
- How long the tick remained attached
75
What are the manifestations of Lyme disease? (4)
- Rash
- Fever
- Joint pain
- Swollen lymph nodes
76
Manifestations of Lyme disease occur ______ after a tick bite
3 - 30 days
77
Neurological deficits occur during what stages of Lyme disease?
2 - 3
78
What neurological deficits are common during stage 2 of Lyme disease? (2)
- Peripheral neuropathy
- Bell's Palsy
79
What is the primary diagnostic test used in the diagnosis of Lyme disease?
2-step blood testing for antibodies
80
Blood testing for diagnosis of Lyme disease must take place ...
After symptoms are present
81
What medications are used for the treatment of Lyme disease?
IV antibiotics - amoxicillin / tetracycline
