TARCUAN, Kawa M.

May 10, 2020

BSRT-2

Myasthenia Gravis

Part I, Case Review.

A. Patient Information

DEMOGRAPHICS:
SCORE:45/50
Age: 35 y/o Gender: Female CONTENT: 23/25 ORGANIZATION: 14/15 MECHANICS: 8/10
COMMENTS: Great work. The case provided is clear and
sufficiently understandable except for some missing pertinent
Race: Spanish-American clinical data. The discussion could have been improved by proper
clinical correlation.
Occupation: School teacher

Status: Married Hobbies: cooking.

C/C: Diplopia, Oral Dysphagia and Muscle Fatigue.

HPI: Excellent HPI

3-week PTA, the patient noticed that her eyes “felt tired”. She began experiencing slight double vision.

Thinking that she was working too hard, she slowed down a bit and went to bed earlier for about a
week.

1-week PTA, she still experienced these symptoms, no consultation was done and no medication was
taken until,

1-day PTA, she progressively felt weaker. Her legs quickly became tired and began having trouble
chewing her food

This concerned her, she immediately sought medical help and after some physical examination and
reviewing the woman’s history, the physician admitted her to the hospital for further evaluation and
treatment.

PMH; Should have included pertinent positive and negative. None

P/E/S/O

The patient is living with her 3-year-old son and an unemployed husband. She works as a school
teacher. Her colleagues at school considered her a nonstop worker. At home, she was always on the
move. She loves cooking, reading, and landscaping. She had never smoked.

B. Clinical Findings

PHYSICAL EXAMINATION:

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General assessment:

- Weak in appearance - No cough

- No pallor - Bilateral pstosis (drooping eyelids)

- No clubbing, no rash - muscle fatigue (lower extremities)

VS:

BP- 132/86 (normal) PR- 90 bpm (normal) RR- 10 breaths per minute (Bradypnic)

Temp- 38oC (rectal temperature) SaO2- 88% (desaturated)

I.P.P.A Could have included other systemic findings as well aside from respiratory

I.+ Ptosis, +weak in appearance, +oral dysphagia, -cyanosis, +Bradypnea.

P.+muscle fatigue, +>2sec. CRT, -mass, -lesion, +regular pulse rhythm.

P.+dull percussion at the left lung.

A.+normal vesicular breath sound over right lung, +diminished to absent breath sound over left lung

C. Diagnostics Assessment

The patient was first tested using the ice pack test which results positive for myasthenia gravis when
her ptosis improved by 5mm. Electromyography was also done to disclose the extensive muscle
involvement and high degree fatiguability in all affected muscles which the results shows that the px is
diagnosed with myasthenia gravis. Pre-ABG was done to check px oxygenation and the result shows
uncompensated respiratory acidosis with moderate hypoxemia at room air ( PH 7.32, PaCO2 51mmHg,
HCO3-23 mEq/L, PaO2 59 mmHg, SaO2 88%RA) which alarmed the R.T to call the physician and
reported an assessment of acute ventilatory failure causing the px to be intubated and hooked to
mechanical ventilator. Post-ABG was done after hooking which shows (pH 7.28, PaCO2 64mmHg,
HCO3-29 mEq/L, PaO2 52 mmHg, SaO2 81%@50% FiO2) partially compensated respiratory acidosis
with uncorrected oxygenation at 50% FiO2. 45 minutes later, a Chest radiograph was taken to check
the E.T placement and was seen to be inserted too far into the patients right mainstem. The E.T tube
was then pulled back 3cm to 20cm at the lip, follow up CXR done confirmed E.T tube was now
appropriately positioned about 2cm above carina. 3 days later, chest radiograph and ABG was again
conducted which revealed a new infiltrate in the right lower lobe consistent with pneumonia or
atelectasis, ABG: pH 7.28, PaCO2 36 mmHg, HCO3-16 mEq/L, PaO2 41mmHg, SaO2 69%
(uncompensated metabolic acidosis with uncorrected oxygenation at 50 FiO2).

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D. Therapeutics, Patient Outcome and Follow-up.

Patient was apparently well until she started having symptoms and was admitted. During her
admission, Patient was given edrophonium to increase muscle strength but only lasted for 10 minutes.
Patient’s condition continued to deteriorate which can be seen in both physical examination and Lab
tests that’s why she was then intubated and was hooked to a mechanical ventilator to support her
breathing, but still her condition worsened. She appears pale and there are now noted large amounts of
thick yellowish sputum suctioned every 30 minutes, no improvement seen in muscular paralysis, course
crackles were auscultated in both lungs and her mechanical ventilator settings were adjusted but still
her ABG results are critical. Follow up plans includes Bronchial hygiene for her secretions with frequent
suctioning, percussion, postural drainage, and possible administration for mucolytics, aerosolized
medication like bronchodilators to narrow the airways.

Des Jardins, T., & Burton, G., (2016). Clinical Manifestation Assessment of Respiratory Disease.

(7th ed). Elsevier: St. Louis Missouri

Kacmarek, R., Stoller, J., & Heuer, A. (2017). Egan’s Fundamentals of Respiratory Care. (11th ed).

Elsevier: Philippines

Part II. Discussion:

A. Epidemiology:

 Affects 140-200/1,000,000 population

 36,000-60,000 Americans
 Women > men before age 50
 Peak onset 20-40 years (women)
 Men more commonly diagnosed ages 60-80

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 The prevalence of myasthenia gravis in the United States is estimated at 14 to 20 per 100,000
population, approximately 36,000 to 60,000 cases in the United States. However, myasthenia
gravis remains underdiagnosed and the prevalence is probably higher. Previous studies have
shown that women are more often affected than men. The most common age at onset is the
second and third decades in women and the seventh and eighth decades in men. As the
population ages, the average age at onset has increased correspondingly, and now males are
more often affected than females, and the onset of symptoms is usually after age 50. A 2015 study
in acetylcholine receptor antibody (AChR-Abs) positive Caucasian has demonstrated that there is
no specific causal gene for myasthenia gravis there are specific regulatory genes that
influence immune regulation. In addition, about 3% of the study population had a primary relative
with myasthenia gravis suggesting a small but distinct but not direct genetic influence.

B. Pathology:

The cause of myasthenia gravis appears to be related to Ach

receptor (AChR) antibodies (IgG antibodies) that block the nerve impulse
transmissions at the neuromuscular junction.

Patients who have detectable antibodies to the AChR, or to the

muscle-specific receptor tyrosine kinase (MuSK), are said to have
seropositive myasthenia gravis, whereas those lacking both AChR and
MuSK antibodies on standard assays are said to have seronegative myasthenia gravis. About 50% of
patients with only ocular myasthenia gravis are seropositive. About 90% of generalized cases of
myasthenia gravis are seropositive. It is believed that the IgG antibodies disrupt the chemical
transmission of ACh at the neuromuscular junction by (1) blocking the ACh from the receptor sites of
the muscular cell, (2) accelerating the breakdown of ACh, and (3) destroying the receptor sites.
Receptor-binding antibodies are present in 85% to 90% of persons with myasthenia gravis. Although
the specific events that activate the formation of the antibodies remain unclear, the thymus gland is
often abnormal; it is generally presumed that the antibodies arise within the thymus or in related tissue.

The hallmark Clinical presentation of myasthenia gravis is chronic muscle fatigue which your
muscle becomes progressively weaker with ptosis, diplopia, DOB, oral dysphagia and weakness in
arms, fingers, legs and neck which is seen the case above. The respiratory muscle of the diaphragm
and the chest wall can also become weak and impair ventilation that’s why the px was hooked to the
mechanical ventilator. Due to the impairment in deep breathing and coughing, this now leads to
excessive bronchial secretions that’s why the px is being suctioned every 30 min.

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C. Diagnostics:

Ice pack test- The test consists of the application of ice pack to the patient’s symptomatic eye for 3 to 5
minutes. The test is considered positive for myasthenia gravis when there is improvement of the ptosis
(an increase of at least 2mm in the palpebral fissure from before to after test. The test was taken by the
px and resulted positive for M.G when her ptosis improved by 5mm.

Electromyography:

Electromyography (EMG) measures muscle response or electrical activity in response to a nerve's

stimulation of the muscle. The test is used to help detect neuromuscular abnormalities. During the test,
one or more small needles (also called electrodes) are inserted through the skin into the muscle.

Edrophonium (Tensilon) Test- Edrophonium is a short acting drug, it blocks cholinesterase from
breaking down Ach after it has been released from the terminal axon.

D. Treatment:

a. Acetylcholinesterase Inhibitors such as pyridostigmine (Mestinon) recommended as the first line of

treatment for symptomatic myasthenia gravis. It increases the concentration of Ach to compete with the
circulating anti-Ach antibodies.

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b. Oxygen therapy- used to correct px hypoxemia, decrease the work of breathing, and decrease
myocardial work.

c. Bronchial hygiene therapy- Because of the excessive mucus production and accumulation
associated with myasthenia gravis, Bronchial hygiene is used to improve the mobility of bronchial
mucus secretions.

d. Lung Expansion therapy- To prevent alveolar consolidation and atelectasis

e. Mechanical Ventilator- provide and support alveolar gas exchange and eventually return the patient
to spontaneous breathing (for sever cases)

Des Jardins, T., & Burton, G., (2016). Clinical Manifestation Assessment of Respiratory Disease.

(7th ed). Elsevier: St. Louis Missouri

https://myasthenia.org/For-Professionals/Clinical-Overview-of-MG

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