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AP Exam 3 > Neuromuscular Diseases Part 2 > Flashcards

Neuromuscular Diseases Part 2 Flashcards

(82 cards)

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1
Q

What disease results in reduced acetylcholine receptors?

A

Myasthenia gravis

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2
Q

What disease’s clinical hallmark is skeletal muscle weakness that worsens with activity and improves with rest?

A

Myasthenia gravis

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3
Q

The severity of the disease directly _______ with the ability of antibodies to decrease the NUMBER OF AVAILABLE ACH RECEPTORS.

A

Correlates

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4
Q

Myasthenia gravis is more common in females or males?

A

Females

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5
Q

What is the age of onset for myasthenia gravis for females? Males?

A

20-30 yo

60-70 yo

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6
Q

What triggers myasthenia gravis?

A

The trigger is unknown.

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7
Q

True or false: 85% of myasthenia gravis patients have detectable antibodies.

A

True

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8
Q

The level of antibodies is directly correlated to severity in myasthenia gravis patients. True or false?

A

False. No correlation.

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9
Q

What other issues do myasthenia gravis patients have?

A

Thymomas

65% have hyperplastic thymus glands.

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10
Q

What are the clinical signs and symptoms of myasthenia gravis? (4)

A

Muscle weakness that worsens with activity

Improves with rest

Head and neck muscles affected early

Trunk and limb muscles affected later

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11
Q

What common physical symptoms do myasthenia gravis patients have? (2)

A

Ptosis

Diplopia

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12
Q

What exacerbates myasthenia gravis? (6)

A 
Vaccinations
Infection
Stress
Pregnancy
Surgery
Temperature changes --heat makes worse
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13
Q

Neonates of myasthenic moms will exhibit transient myasthenia gravis. True or false?

A

True.

It may last 1 to 3 weeks.

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14
Q

What is the treatment for myasthenia gravis? (5)

A 
Anti-cholinesterase drugs like Neo
Thymectomy
Steroids
Immunosuppressants
Plasmapheresis to remove antibodies
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15
Q

If the patient has myasthenia gravis what is the risk or anesthetic concern if there is bulbar involvement? I.e. Can they stick out their tongue? Can they show their teeth?

A

Risk of aspiration!

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16
Q

What if myasthenia gravis has respiratory weakness? What is the anesthetic consideration?

A

They may need post op ventilation.

Pulmonary function tests may also be useful.

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17
Q

What are anesthetic considerations for myasthenia gravis patients? (4)

A

Risk of aspiration
Postop ventilation
Regional versus general anesthesia
Muscle relaxants

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18
Q

Myasthenia gravis have a _______ response to succinylcholine.

A

Decreased

Remember succinylcholine acts like acetylcholine so there is a lower response to succinylcholine.

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19
Q

The effective dose of succinylcholine will be ___ times normal in myasthenia gravis patients.

A

2

ED95 is 2.6

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20
Q

When giving nondepolarizing muscle relaxants to myasthenia gravis patients, what do you expect to happen?

A

Prolonged effects

Use much smaller dose or none at all if possible.

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21
Q

Use caution with neuromuscular blocker reversal with myasthenia gravis patients. True or false?

A

True

May want to consider Cis-atricurium to avoid using reversal.

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22
Q

In myasthenia gravis patients, what other drugs in conjunction with NMBs will potentiate block even more? (3)

A

Inhaled anesthetics
Magnesium
Aminoglycosides like gentamyacin

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23
Q

What are indicated risk factors for post op mechanical ventilation in myasthenia gravis patients? (4)

A

MG greater than six years
Additional chronic respiratory disease
Pyridostigmine dose > 750 mg/day
Preop vital capacity< 2.9 L

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24
Q

What are the causes of myasthenic crisis? (2)

A

Disease exacerbation

Inadequate drug therapy

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25
Myasthenic crisis must be differentiated from cholinergic crisis which is caused by excessive anticholinesterase administration. True or false?
True
26
How do we differentiate a myasthenic crisis from a cholinergic crisis?
Tensilon test
27
What is Tensilon?
A short acting anti-cholinesterase
28
What does a negative result in a Tensilon test indicate for myasthenic patients? Positive result?
Postop ventilation is required. They get a little better.
29
Disease where antibodies attack Ca gated channel at presynaptic nerve terminals to decrease the release of ACH at NMJ
Myasthenic syndrome also known as Lambert Eaton syndrome.
30
What disease is associated with: Small cell lung cancer Proximal limb muscle weakness Affects legs more than arms Repeated effort improves muscle activity Anti cholinesterase drugs have no benefit Sensitive to both depolarizing and nondepolarizing muscle relaxants
Myasthenic syndrome
31
What is Lou Gehrig's disease?
Amyotrophic lateral sclerosis
32
What is the most common progressive motor neuron disease?
Lou Gehrig's disease
33
Lou Gehrig's disease affects more females than males and less than 55 years of age. True or false?
False More males over 55 years of age.
34
Death usually results from what in Lou Gehrig's disease?
Pulmonary infections
35
What disease is a loss of nerve cells in anterior horn of spinal cord and lower brainstem?
Lou Gehrig's disease
36
What disease affects motor tracts and spares sensory tracts?
Lou Gehrig's disease
37
What disease involves upper and lower motor neurons?
Lou Gehrig's disease or ALS
38
What muscles are affected in the late stages of Lou Gehrig's disease or ALS?
Pharyngeal laryngeal and respiratory muscles.
39
Heart is unaffected but ANS is affected in Lou Gehrig's disease, or ALS. True or false?
True
40
What is a test to indicate a person has ALS or Lou Gehrig's disease?
Positive Babinski sign
41
What are signs of ALS? (5)
``` Hyperactive reflexes Orthostatic hypotension Positive Babinski sign Atrophy Weakness in limbs ```
42
ALS patients are a high aspiration risk. True or false?
True
43
Most ALS patients have normal PFTs. True or false?
False
44
Use succinylcholine in ALS patient. True or false?
False.
45
ALS patients are sensitive to nondepolarizing agents. True or false?
True
46
Use regional if possible in ALS patients. True or false?
True
47
What is a demyelinating disease of brain and spinal cord in CNS?
Multiple sclerosis
48
What nerves are generally not affected in multiple sclerosis?
Peripheral nerves
49
MS is an autoimmune disease. True or false?
True
50
MS is more common in males. True or false?
False Two times more common in females
51
What forms in patients with MS?
De-myelinated plaques
52
What is the initial presentation of MS? (5)
``` Weakness Numbness Tingling in limb Disequilibrium Double vision ```
53
At what age does MS usually present?
20 to 40 years old
54
There is a relapsing and remitting course in MS. True or false?
True
55
What are MS relapses associated with? (5)
``` Surgery Anesthesia Fever Trauma Pregnancy ```
56
An increase as little as .5°C can completely block conduction in a multiple sclerosis patient. True or false?
True | Do not overheat these patients
57
What are anesthetic considerations for MS patients? (4)
Avoid sux Avoid temperature increases Be prepared for autonomic lability Steroid supplementation
58
Demyelinated neurons are more susceptible to toxicity thus... (3)
Avoid using lidocaine Avoid spinals Epidurals OK
59
What neuromuscular disease results in muscular weakness and wasting without denervation?
Muscular dystrophy
60
Is muscular dystrophy painful?
No
61
What is the source of muscular dystrophy?
Abnormality of dystrophin
62
What is the most common form of muscular dystrophy?
Duchenne's
63
Is muscular dystrophy more common in females or males?
Males X-linked recessive
64
When does muscular dystrophy usually present?
3 to 5 years of age
65
What disease is characterized by proximal muscle weakness?
Muscular dystrophy
66
What are the consequences of muscular dystrophy? (5)
``` Pseudohypertrophy of calf muscles Abnormal gait Intellectual impairment Small shoulders Severe kyphoscoliosis ```
67
Death is usually by ___-____ y/o due to what 2 reasons with MD patients?
20-30 | CHF and pneumonia
68
Why will you normally see muscular dystrophy patient?
Muscle biopsy | Spine surgery
69
What are muscular dystrophy patients at risk for?
Aspiration | Pneumonia
70
What cardiac issues do muscular dystrophy patients have?
Mitral regurgitation Cardiac myopathy Pulmonary hypertension due to sleep apnea
71
Do use succinylcholine for muscular dystrophy patients. True or false?
False Can cause hyperkalemia leading to cardiac arrest.
72
There is an increased incidence of MH in MD patients. True or false?
True
73
What are types of muscular dystrophy? (4)
Beckers--less severe Facioscapulohumeral dystrophy Limb-girdle dystrophy--slow progressive Duchenne's
74
Avoid succinylcholine in all cases of muscular dystrophy. True or false?
True
75
What is an acute demyelinating polyneuropathy?
Guillian barre syndrome
76
Guillian barre syndrome is a sudden onset of ____ motor paralysis.
Ascending
77
Guillian Barre is thought to be immune mediated against myelin of ________ nerves.
Peripheral
78
Guillian barre usually follows _____ infections.
Viral | ...of GI and respiratory
79
Guillian barre usually seen with what other diseases?
Hodgkins | HIV
80
Prognosis is good usually with a complete recovery in Guillian Barre patients. True or false?
True
81
What are anesthetic considerations of Guillian barre patients? (4)
Aspiration due to bulbar muscle involvement Avoid succinylcholine Regional is controversial Autonomic lability
82
What is elevated in MD patients?
creatine kinase

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