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AP Exam 3 > Neuromuscular Diseases Part 2 > Flashcards

Neuromuscular Diseases Part 2 Flashcards

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
Q

Myasthenic crisis must be differentiated from cholinergic crisis which is caused by excessive anticholinesterase administration. True or false?

A

True

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26
Q

How do we differentiate a myasthenic crisis from a cholinergic crisis?

A

Tensilon test

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27
Q

What is Tensilon?

A

A short acting anti-cholinesterase

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28
Q

What does a negative result in a Tensilon test indicate for myasthenic patients?

Positive result?

A

Postop ventilation is required.

They get a little better.

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29
Q

Disease where antibodies attack Ca gated channel at presynaptic nerve terminals to decrease the release of ACH at NMJ

A

Myasthenic syndrome also known as Lambert Eaton syndrome.

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30
Q

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

A

Myasthenic syndrome

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31
Q

What is Lou Gehrig’s disease?

A

Amyotrophic lateral sclerosis

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32
Q

What is the most common progressive motor neuron disease?

A

Lou Gehrig’s disease

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33
Q

Lou Gehrig’s disease affects more females than males and less than 55 years of age. True or false?

A

False

More males over 55 years of age.

34
Q

Death usually results from what in Lou Gehrig’s disease?

A

Pulmonary infections

35
Q

What disease is a loss of nerve cells in anterior horn of spinal cord and lower brainstem?

A

Lou Gehrig’s disease

36
Q

What disease affects motor tracts and spares sensory tracts?

A

Lou Gehrig’s disease

37
Q

What disease involves upper and lower motor neurons?

A

Lou Gehrig’s disease or ALS

38
Q

What muscles are affected in the late stages of Lou Gehrig’s disease or ALS?

A

Pharyngeal laryngeal and respiratory muscles.

39
Q

Heart is unaffected but ANS is affected in Lou Gehrig’s disease, or ALS. True or false?

40
Q

What is a test to indicate a person has ALS or Lou Gehrig’s disease?

A

Positive Babinski sign

41
Q

What are signs of ALS? (5)

A 
Hyperactive reflexes
Orthostatic hypotension
Positive Babinski sign
Atrophy
Weakness in limbs
42
Q

ALS patients are a high aspiration risk. True or false?

43
Q

Most ALS patients have normal PFTs. True or false?

44
Q

Use succinylcholine in ALS patient. True or false?

45
Q

ALS patients are sensitive to nondepolarizing agents. True or false?

46
Q

Use regional if possible in ALS patients. True or false?

47
Q

What is a demyelinating disease of brain and spinal cord in CNS?

A

Multiple sclerosis

48
Q

What nerves are generally not affected in multiple sclerosis?

A

Peripheral nerves

49
Q

MS is an autoimmune disease. True or false?

50
Q

MS is more common in males. True or false?

A

False

Two times more common in females

51
Q

What forms in patients with MS?

A

De-myelinated plaques

52
Q

What is the initial presentation of MS? (5)

A 
Weakness
 Numbness
Tingling in limb
Disequilibrium
Double vision
53
Q

At what age does MS usually present?

A

20 to 40 years old

54
Q

There is a relapsing and remitting course in MS. True or false?

55
Q

What are MS relapses associated with? (5)

A 
Surgery
Anesthesia
Fever
Trauma
Pregnancy
56
Q

An increase as little as .5°C can completely block conduction in a multiple sclerosis patient. True or false?

A

True

Do not overheat these patients

57
Q

What are anesthetic considerations for MS patients? (4)

A

Avoid sux
Avoid temperature increases
Be prepared for autonomic lability
Steroid supplementation

58
Q

Demyelinated neurons are more susceptible to toxicity thus… (3)

A

Avoid using lidocaine
Avoid spinals
Epidurals OK

59
Q

What neuromuscular disease results in muscular weakness and wasting without denervation?

A

Muscular dystrophy

60
Q

Is muscular dystrophy painful?

61
Q

What is the source of muscular dystrophy?

A

Abnormality of dystrophin

62
Q

What is the most common form of muscular dystrophy?

A

Duchenne’s

63
Q

Is muscular dystrophy more common in females or males?

A

Males

X-linked recessive

64
Q

When does muscular dystrophy usually present?

A

3 to 5 years of age

65
Q

What disease is characterized by proximal muscle weakness?

A

Muscular dystrophy

66
Q

What are the consequences of muscular dystrophy? (5)

A 
Pseudohypertrophy of calf muscles
Abnormal gait
Intellectual impairment
Small shoulders
Severe kyphoscoliosis
67
Q

Death is usually by ___-____ y/o due to what 2 reasons with MD patients?

A

20-30

CHF and pneumonia

68
Q

Why will you normally see muscular dystrophy patient?

A

Muscle biopsy

Spine surgery

69
Q

What are muscular dystrophy patients at risk for?

A

Aspiration

Pneumonia

70
Q

What cardiac issues do muscular dystrophy patients have?

A

Mitral regurgitation
Cardiac myopathy
Pulmonary hypertension due to sleep apnea

71
Q

Do use succinylcholine for muscular dystrophy patients. True or false?

A

False

Can cause hyperkalemia leading to cardiac arrest.

72
Q

There is an increased incidence of MH in MD patients. True or false?

73
Q

What are types of muscular dystrophy? (4)

A

Beckers–less severe
Facioscapulohumeral dystrophy
Limb-girdle dystrophy–slow progressive
Duchenne’s

74
Q

Avoid succinylcholine in all cases of muscular dystrophy. True or false?

75
Q

What is an acute demyelinating polyneuropathy?

A

Guillian barre syndrome

76
Q

Guillian barre syndrome is a sudden onset of ____ motor paralysis.

77
Q

Guillian Barre is thought to be immune mediated against myelin of ________ nerves.

A

Peripheral

78
Q

Guillian barre usually follows _____ infections.

A

Viral

…of GI and respiratory

79
Q

Guillian barre usually seen with what other diseases?

A

Hodgkins

HIV

80
Q

Prognosis is good usually with a complete recovery in Guillian Barre patients. True or false?

81
Q

What are anesthetic considerations of Guillian barre patients? (4)

A

Aspiration due to bulbar muscle involvement
Avoid succinylcholine
Regional is controversial
Autonomic lability

82
Q

What is elevated in MD patients?

A

creatine kinase

AP Exam 3 (5 decks)

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