Myasthenia gravis is a neuromuscular disorder characterized by variable weakness

of voluntary muscles, which often improves with rest and worsens with activity. The
condition is caused by an abnormal immune response.

CAUSE:

In myasthenia gravis, weakness occurs when the nerve impulse to initiate or sustain
movement does not adequately reach the muscle cells. This is caused when
immune cells target and attack the body's own cells (an autoimmune response

RISK FACTORS:

female gender with ages 20 – 40

familial myasthenia gravis

D-penicillamine ingestion (drug induced myasthenia)

having other autoimmune diseases like Rheumatoid arthritis and Lupus

SIGNS AND SYMPTOMS

• Muscle weakness, including:

o Swallowing difficulty, frequent gagging, or choking
o Paralysis
o Muscles that function best after rest
o Drooping head
o Difficulty climbing stairs
o Difficulty lifting objects
o Need to use hands to rise from sitting positions
o Difficulty talking
o Difficulty chewing
• Vision problems:
o Double vision
o Difficulty maintaining steady gaze
o Eyelid drooping
o
Additional symptoms that may be associated with this disease:
• Hoarseness or changing voice
• Fatigue
• Facial paralysis
• Drooling
• Breathing difficulty
DIAGNOSTIC TESTS:

Blood tests

If the diagnosis is suspected, serology can be performed in a blood test to identify

certain antibodies:

 One test is for antibodies against the acetylcholine receptor. The test has a
reasonable sensitivity of 80–96%, but in MG limited to the eye muscles (ocular
myasthenia) the test may be negative in up to 50% of the cases.

 A proportion of the patients without antibodies against the acetylcholine

receptor have antibodies against the MuSK protein.[11]

Neurophysiology

The repetitive nerve stimulation test; in single fiber electromyography, which is

considered to be the most sensitive (although not the most specific) test for MG, a
thin needle electrode is inserted into a muscle to record the electric potentials of
individual muscle fibers. By finding two muscle fibers belonging to the same motor
unit and measuring the temporal variability in their firing patterns (i.e. their 'jitter'),
the diagnosis can be made.
Edrophonium test

The "edrophonium test" is infrequently performed to identify MG; its application is

limited to the situation when other investigations do not yield a conclusive
diagnosis. This test requires the intravenous administration of edrophonium
chloride (Tensilon, Reversol) or neostigmine (Prostigmin), drugs that block the
breakdown of acetylcholine by cholinesterase (cholinesterase inhibitors) and
temporarily increases the levels of acetylcholine at the neuromuscular junction. In
people with myasthenia gravis involving the eye muscles, edrophonium chloride will
briefly relieve weakness.
Imaging
A chest X-ray is frequently performed; it may point towards alternative diagnoses
(e.g. Lambert-Eaton due to a lung tumor) and comorbidity. It may also identify
widening of the mediastinum suggestive of thymoma, but computed
tomography (CT) or magnetic resonance imaging (MRI) are more sensitive ways to
identify thymomas, and are generally done for this reason.

Pulmonary function test

Spirometry (lung function testing) may be performed to assess respiratory function

if there are concerns about a patient's ability to breathe adequately. The
forced vital capacity may be monitored at intervals in order not to miss a gradual
worsening of muscular weakness.
Pathological findings

Muscle biopsy is only performed if the diagnosis is in doubt and a muscular

condition is suspected. Immunofluorescence shows IgG antibodies on the
neuromuscular junction.

TREATMENT

Medication

Neostigmine, chemical structure.

 Acetylcholinesterase inhibitors: neostigmine and pyridostigmine can improve

muscle function by slowing the natural enzyme cholinesterase that
degrades acetylcholine in the motor end plate;

Azathioprine, chemical structure.

 Immunosuppressive drugs: prednisone, cyclosporine, mycophenolate

mofetil and azathioprine may be used. It is common for patients to be treated
with a combination of these drugs with a cholinesterase inhibitor.

Plasmapheresis and IVIG

If the myasthenia is serious (myasthenic crisis), plasmapheresis can be used to

remove the putative antibody from the circulation. Also, Intravenous
immunoglobulins (IVIG) can be used to bind the circulating antibodies.
Surgery
Thymectomy

Thymectomy, the surgical removal of the thymus, is essential in cases

of thymoma in view of the potential neoplastic effects of the tumor.
 PATHOPHYSIOLOGY

B cells

Plasma cells

Secretion of IgG antibodies

(against acetylcholine receptors)

Fixation of antibodies at receptor sites

ACh receptor sites, weakend or destroyed

by attached antibodies, block ACh reception

Depolarization and muscle contraction

do not occur, Neuromuscular transmission is blocked

Class I: Any eye Class II: Eye muscle weakness of Class III: Eye muscle Class IV: Eye muscle
any severity, mild weakness of
Class V:
muscle weakness, weakness of any severity, weakness of any severity,
other muscles moderate weakness of other severe weakness of other
Intubation
possible ptosis, muscles muscles needed to
no other evidence maintain airway
Class IIa: Predominantly limb or
of muscle weakness axial muscles Class IIIa: Predominantly Class IVa: Predominantly
elsewhere limb or axial muscles limb or axial muscles
Class IIb: Predominantly bulbar
and/or respiratory muscles Class IIIb: Predominantly Class IVb: Predominantly
bulbar and/or respiratory bulbar and/or respiratory
muscles muscles.
NURSING DIAGNOSIS:

Ineffective Airway Clearance

Assist to a sitting position with head slightly flexed, shoulders relaxed, and kneed flexed.

- Lying flat causes the abdominal organs to shift toward the chest, crowding the lungs and making it
more difficult to breath.

Encourage her to take several deep breaths

- Deep breathing promotes oxygenation before controlled coughing.

Encourage her to take a deep breath, hold for 2 seconds, and cough 2 or 3 times in succession

- Controlled coughing is accomplished by the closure of the glottis and explosive expulsion of air from
the lungs by the work of abdominal and chest muscles.

Promote systemic fluid hydration

- Adequate fluid intake enhances liquefaction of pulmonary secretions and facilitates expectoration of
mucus.

Impaired Physical Mobility

Provided progressive mobilization by maintaining head of bed at least 30 degree angle and assisted the client slowly
from lying to sitting position.

- Prolonged bed rest can cause a sudden drop in blood pressure (orthostatic hypotension) as blood
returns to peripheral circulation.

Keep the patient’s head and neck in neutral position

 - This position promotes venous drainage from the brain and decreases ICP

Avoid unnecessary care activities.

- Frequent stimulation of the patient increases brain activity and ICP. Clustering care activities in a short
period of time also increases ICP.

Perform passive ROM at least three to four times daily. Exercises are done slowly to allow the muscles time to relax,
and support the extremity above and below the joint to prevent strain on joints and tissues. Stop when the pain and
resistance is met.

- A voluntary muscle will lose tone and strength and becomes shortened from reduced range of motion
or lack of exercise.

Risk for Aspiration

Maintain upright position for 30 to 45 minutes after feeding

- The upright position facilitates the gravitational flow of food or fluid through the alimentary tract. If the head of
the bed cannot be elevated because of the patient’s condition, use a right side-lying position after feedings to
facilitate passage of stomach contents into the duodenum.

Position patient at 90-degree angle, whether in bed or in a chair or wheelchair. Use cushions or pillows to maintain position

- Proper positioning of patients with swallowing difficulties is of primary importance during feeding or eating.

Offer foods with consistency that patient can swallow. Use thickening agents as appropriate. Cut foods into small pieces

- Semisolid foods like pudding and hot cereal are most easily swallowed. Liquids and thin foods like creamed soups
are most difficult for patients with dysphagia.
Encourage patient to chew thoroughly and eat slowly during meals. Instruct patient not to talk while eating