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Mayasthenia gravis

Myasthenia Gravis NCLEX Review

• What is Myasthenia Gravis? It’s an autoimmune condition


where the body attacks the receptors that allow for
voluntary muscle control, which leads to muscle weakness.
• What voluntary muscles are involved?
• Eyes: most likely will be the first sign something is wrong
• Throat: another common sign…hoarse voice and problems
swallowing
• Face
• Arms/Legs
• Respiratory muscles for breathing (in severe cases)
• In myasthenia gravis the issue arises at the neuromuscular junction. This is the
place where the motor neuron and muscle fiber meet and the neurotransmitter
acetylcholine is released.
• At this junction are receptors on the muscle fiber called nicotinic acetylcholine
receptors that become stimulated when acetylcholine is released and cause
contraction of the muscle fiber.
• However, in myasthenia gravis the nicotinic acetylcholine receptors are being
attacked by antibodies the immune system has created and are not working
properly. These receptors can NOT access the release of acetylcholine and cause
muscle contraction, so there is muscle weakness.
• The thymus gland (found anteriorly in the upper part of the chest behind the
sternum in between the lungs) plays a very important role in our immune system
health. It’s the creator of T-cells, which are super important for immune health by
fighting viruses and bacteria.
• In children the thymus is large (remember it will
produce most of the body’s T-cells by the time a
person attains puberty). It should shrink by
adulthood and then in older age be like a fatty tissue.
But in people with myasthenia gravis, the thymus
gland tends to stay large and develops tumors or
collection of immune cells in the gland. It is thought
that the thymus erroneously causes immune cells to
produce antibodies that will attack receptor sites on
these voluntary muscle neuromuscular junctions.
Complications of Myasthenia Gravis

• Myasthenia gravis can go into remission where the patient


doesn’t have signs and symptoms, but some patients can
experience severe acute exacerbation with a complication
called Myasthenic Crisis. This is where the disease is becoming
worse and the patient may need intubation and mechanical
ventilation to breathe.
• Risk factors for experiencing Myasthenic Crisis: improper dosage
(not enough) of anticholinesterase meds to treat this condition,
stress (physical…surgery or mental), and respiratory infection.
• That patient will have severe weakness of muscles that cause
respiratory failure.  
• How is it diagnosed?
• Edrophonium (Tensilon test): During this test an
anticholinesterase medication is given called
Edrophonium.
– How does the medication work? It prevents the breakdown of
acetylcholine at the neuromuscular junction, which allows more
of the neurotransmitter to be present at the neuromuscular
junction.
– It is used to diagnose Myasthenia Gravis AND differentiate
between a myasthenic crisis and cholinergic crisis (see more
about cholinergic crisis in the pharmacology part of this lecture).
IMPORTANT:

• What will happen if Edrophonium is given to a patient with


myasthenia gravis or to a patient in myasthenic crisis? The
patient’s signs and symptoms will temporarily improve. Hence,
the test is positive.
• What will happen if Edrophonium is given to a patient with
cholinergic crisis? The patient’s signs and symptoms will become
worse….NO improvement. Hence, the test is negative.
• If this happens the nurse (per MD order) will administer the
antidote for Edrophonium, which is ATROPINE! It is also
important to have the patient on a cardiac monitor during the
test and a crash cart within reach if an emergency arises.
• Nursing Interventions & Medications for Myasthenia Gravis
• Monitor respiratory status: effort of breathing, respiratory rate, oxygen
saturation, does the patient feel like it’s hard to breathe? If hospitalized with
exacerbation needs resuscitation equipment close by: suction etc.
• Monitor neuromuscular status:
• Assessing vision (double vision?), quality of voice, swallowing (important for
medication administration…ALWAYS check swallowing before giving meds),
facial appearance, strength with arms/legs, cranial nerves
• Safety: at risk for injury….needs assistance at all times (remember the patient
may have double vision (eye patch to help with this), arm and leg weakness)
• Speech pathologist will evaluate and recommend treatment based on findings.
Patient may need or have a feeding tube and you will be managing this as the
nurse.
• Monitor for aspiration, hob of greater than 30′ while eating
• Education about food because patient may have difficulty chewing and swallowing:
• smaller meals that are easy to chew foods…pick food options that are soft and
require minimal effort to chew
• try scheduling meals during peak time of medication (30 minutes to 1 hour before
eating) so chewing and swallowing muscle will have the most access to
acetylcholine
• Thicken liquids for swallowing
• Take small bites and rest while eating and before a meal
• It’s best to have large meals at the beginning of the day than at the end (remember
patients with MG becomes progressively weaker throughout the day)
• Educate about performing most of their activities early in the day when the patient
will have the most energy.
• Help patient identify things that makes symptoms worst: menstrual cycle, sickness,
stress, extreme temperatures
Signs and Symptoms of Cholinergic Crisis:

• Think of the signs and symptoms experienced during cholinergic crisis similar to the 
parasympathetic nervous system being in overdrive:
• Pupil constriction
• Bronchoconstriction….eventually respiratory failure
• Increased salivation and mucous production
• GI problems: abdominal cramping, vomiting, diarrhea
• Bladder incontinence
• Bradycardia
• Treatment: No anticholinesterase medications and atropine to reverse symptoms.
• Other drugs used to treat MG: corticosteroids and immunosuppressants
• Other treatments for MG:
• Thymectomy: removal of the thymus gland (improves symptoms in some patients…NOT a cure)
• Plasmapheresis with administration of IV immunoglobulins (severe cases): filters blood by
removing antibodies that attack the receptors at the neuromuscular junction (temporary
improvement for about 4 weeks to a couple of months….NOT a cure).
Cholinergic vs myasthenia crisis
1.  You’re educating a patient about the
pathophysiology of myasthenia gravis. While
explaining the involvement of the thymus gland,
the patient asks you where the thymus gland is
located. You state it is located?
• A. behind the thyroid gland
• B. within the adrenal glands
• C. behind the sternum in between the lungs
• D. anterior to the hypothalamus
• The answer is C. The thymus is located
anteriorly in the upper part of the chest
behind the sternum in between the lungs.
2. A patient with myasthenia gravis will be eating lunch
at 1200. It is now 1000 and the patient is scheduled
to take Pyridostigmine. At what time should you
administer this medication so the patient will have
the maximum benefit of this medication?
• A. As soon as possible
• B. 1 hour after the patient has eaten (at 1300)
• C. 1 hour before the patient eats (at 1100)
• D. at 1200 right before the patient eats
• The answer is C. Pyridostigmine is an anticholinesterase
medication that will help improve muscle strength. It is important
the patient has maximum muscle strength while eating for the
chewing and swallowing process. Therefore, the medication
should be given 1 hour before the patient eats because this
medication peaks (has the maximum effect) at approximately 1
hour after administration. How does the medication improve
muscle strength? It does this by preventing the breakdown of
acetylcholine. Remember the nicotinic acetylcholine receptors are
damaged and the patient needs as much acetylcholine as possible
to prevent muscle weakness. Therefore, this medication will allow
more acetylcholine to be used…hence improving muscle strength.
3. The neurologist is conducting a Tensilon test (Edrophonium) at the
bedside of a patient who is experiencing unexplained muscle
weakness, double vision, difficulty breathing, and ptosis. Which
findings after the administration of Edrophonium would represent
the patient has myasthenia gravis?
• A. The patient experiences worsening of the muscle weakness.
• B. The patient experiences wheezing along with facial flushing.
• C. The patient reports a tingling sensation in the eyelids and
sudden ringing in the ears.
• D. The patient experiences improved muscle strength.
• The answer is D. During a Tensilon test
Edrophonium is administered. This medication
prevents the breakdown of acetylcholine, which
will allow more of the neurotransmitter
acetylcholine to be present at the neuromuscular
junction….hence IMPROVING muscle strength IF
myasthenia gravis is present. Therefore, if a
patient with MG is given this medication they will
have improved muscle strength.
4. You’re preparing to help the neurologist with
conducting a Tensilon test. Which antidote will
you have on hand in case of an emergency?
• A. Atropine
• B. Protamine sulfate
• C. Narcan
• D. Leucovorin
• The answer is A. Atropine will help reverse the
effects of the drug given during a Tensilon test,
which is Edrophonium, in case an emergency
arises. Edrophonium is a short-acting
cholinergic drug, while atropine is an
anticholinergic.
5. Which patient below is MOST at risk for developing a
cholinergic crisis?
• A. A patient with myasthenia gravis is who is not receiving
sufficient amounts of their anticholinesterase medication.
• B. A patient with myasthenia gravis who reports not
taking the medication Pyridostigmine for 2 weeks.
• C. A patient with myasthenia gravis who is experiencing a
respiratory infection and recently had left hip surgery.
• D. A patient with myasthenia gravis who reports taking
too much of their anticholinesterase medication.
• The answer is D. Remember patients who experience a
cholinergic crisis are most likely to because they’ve
received too much of their anticholinesterase medications
(example Pyridostigmine). However, on the other hand,
patients who have received insufficient amount of their
anticholinesterase medication or have experienced an
illness/stress/surgery are most likely to experience a
myasthenia crisis. Both conditions will lead to muscle
weakness and respiratory failure but from different
causes, which is why a Tensilon test is used to help
differentiate between the two conditions.
6. Which meal option would be the most
appropriate for a patient with myasthenia
gravis?
• A. Roasted potatoes and cubed steak
• B. Hamburger with baked fries
• C. Clam chowder with mashed potatoes
• D. Fresh veggie tray with sliced cheese cubes
• The answer is C. Patients with MG have weak
muscles and this can include the muscles that
are used for chewing and swallowing. The
patient should choose meal options that
require the least amount of chewing and that
are easy to swallow. Option C is a thick type of
soup and the mashed potatoes are soft….both
are very easy to eat and swallow compared to
the other options.
7. Select all the signs and symptoms below that can present
in myasthenia gravis:
• A. Respiratory failure
• B. Increased salivation
• C. Diplopia
• D. Ptosis
• E. Slurred speech
• F. Restlessness
• G. Mask-like appearance of looking sleepy
• H. Difficulty swallowing
• The answers are A, C, D, E, F (restlessness
from hypoxia, which is experienced with
respiratory failure), G, and H.
8. You’re providing teaching to a group of
patients with myasthenia gravis. Which of the
following is not a treatment option for this
condition?
• A. Plasmapheresis
• B. Cholinesterase medications
• C. Thymectomy
• D. Corticosteroids
• The answer is B. These medications are not
used to treat MG, but ANTIcholinesterase
medications (like Pyridostigmine) are used to
treat this condition.
9.  A patient is experiencing unexplained muscle
weakness and respiratory failure. The neurologist
conducts a Tensilon test to differentiate between
myasthenic crisis or cholinergic crisis. During the
test the patient experiences improved muscle
strength. Based on this finding the patient has:
A. Myasthenic Crisis
B. Cholinergic Crisis
C. Neither
• The answer is A. During a Tensilon test the
medication Edrophonium is given, which is an
anticholinesterase medication. This will increase the
availability of acetylcholine at the neuromuscular
junction. If the patient had a cholinergic crisis the
problem is not with the availability of acetylcholine,
but because there is TOO MUCH of it at the
receptor site. Therefore, during this test the patient
would experience worsening of symptoms NOT
improvement.
10. You’re examining a patient’s health history and
find that the patient experienced a cholinergic
crisis last year. As the nurse you know that the
most common cause of a cholinergic crisis is?
• A. Over usage of adrenergic blocker medications
• B. Stress
• C. Respiratory infections
• D. Overmedication of an anticholinesterase
medication
• The answer is D. The most common cause of a
cholinergic crisis is overmedication of an
anticholinesterase medication. However, on
the other hand, the cause of a myasthenic
crisis is NOT enough of an anticholinesterase
medication or having a respiratory infection or
experiencing stress of some type.
11. While assisting with a Tensilon test you will
have what antidote on hand?
• A. Narcan
• B. Atropine
• C. Flumazenil
• D. Glucagon
• The answer is B. Atropine is the antidote for
Edrophonium, which is given during a Tensilon
test.
12. During a Tensilon test the patient has worsening
of muscle weakness. The nurse anticipates that
the physician will give the following order?
• A. Administer Atropine along with an
anticholinesterase medication.
• B. Administer Atropine and hold any further doses
of Pyridostigmine.
• C. Administer Pyridostigmine and hold Atropine.
• D. Administer Edrophonium along with Atropine.
• The answer is B. If a patient experiences
worsening of muscle weakness during a Tensilon
test the patient has cholinergic crisis. Therefore,
the nurse could anticipate that the physician will
order Atropine (to reverse the signs and
symptoms…because remember this drug is the
antidote for Edrophonium which is given during
the test) and to HOLD any further doses of
Pyridostigmine (this is an anticholinesterase drug).
13. Select all the signs and symptoms associated with
cholinergic crisis:
• A. Miosis
• B. Dry mouth
• C. Blurred vision
• D. Constipation
• E. Muscle fasciculation
• F. Diarrhea and abdominal cramping
• G. Respiratory failure
• The answers are A, C, E, F, and G.
14. You’re patient is recovering from a myasthenic crisis and you are
providing education to the patient about the causes of this
condition. Which statement by the patient demonstrates they
understood the teaching about how to prevent this condition?
A. “I will make sure I don’t take too much of my anticholinesterase
medication because it can lead to this condition.”
B. “I will avoid milk products while taking Pyridostigmine because it
increases the chances of toxicity.”
C. “I will avoid taking over-the-counter supplements that contain
aconite.
D. “I will avoid people who are sick with respiratory infections and
be sure not to miss my scheduled doses of Pyridostigmine.”
• The answer is D. Myasthenic crisis is caused by
not enough anticholinesterase medication
(pyridostigmine) or respiratory infection/stress
etc. The other options are NOT causes of this
condition.

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