MYASTHENIA GRAVIS

MYASTHENIA GRAVIS
▪ PRODUCES SPORADIC BUT PROGRESSIVE WEAKNESS AND ABNORMAL
FATIGUE IN STRIATED (SKELETAL) MUSCLES.
▪ THIS WEAKNESS AND FATIGUE ARE EXACERBATED BY EXERCISE AND REPEATED
MOVEMENT BUT IMPROVED BY ANTICHOLINESTERASE DRUGS.
▪ USUALLY, MYASTHENIA GRAVIS AFFECTS MUSCLES INNERVATED BY THE
CRANIAL NERVES (FACE, LIPS, TONGUE, NECK, AND THROAT), BUT IT CAN
AFFECT ANY MUSCLE GROUP.
► Myasthenia gravis has an unpredictable course that includes periods of
exacerbation and remission.
► There’s no known cure.
► Drug treatment has improved the prognosis and allows patients to lead
relatively normal lives, except during exacerbations.
► if the disease involves the respiratory system, it can be life-threatening.
► Myasthenia gravis affects 2 to 20 people per 100,000. I
► It’s most common in women between ages 18 and 25 and men between
ages 50 and 60
What causes it
▪ The cause of myasthenia gravis isn’t known
▪ it commonly accompanies autoimmune and thyroid disorders.
▪ 15% of all patients with myasthenia gravis have thymomas.
Pathophysiology: What happens in myasthenia gravis
✔ The patient’s blood cells and thymus gland produce antibodies that
block, destroy, or weaken the neuroreceptors that transmit nerve
impulses, causing a failure in the transmission of nerve impulses at the
neuromuscular junction.

✔ During normal neuromuscular transmission, a motor nerve impulse

travels to a motor nerve terminal, stimulating the release of a
chemical neurotransmitter called acetylcholine (ACh). When ACh
diffuses across the synapse, receptor sites in the motor end plate react
and depolarize the muscle fiber. The depolarization spreads through
the muscle fiber, causing muscle contraction.
✔ In myasthenia gravis, antibodies attach to the ACh receptor sites. They
block, destroy, and weaken these sites, leaving them insensitive to
ACh, thereby blocking neuromuscular transmission
What to look for :
1. Ocular form – only eye muscles are involved
✔ Diplopia
✔ Ptosis – drooping of eyelids
2. Generalized form – weakness of the muscles of the face and throat
(bulbar symptoms), limb and respiratory weakness
✔ Facial muscle weakness – bland/masklike facial expression
✔ Laryngeal involvement – dysphonia (voice impairment)
✔ Dysphagia (difficulty swallowing) – increases the risk of choking and
aspiration (pharyngeal involvement)
✔ Generalized weakness of the extremities, and intercostal muscles🡪
decreased vital capacity, and respiratory failure => MYASTHENIC CRISIS
 DIAGNOSTIC EVALUATION
1. Acetylcholinesterase inhibitor test (Tensilon test)
✔ The Tensilon test confirms the diagnosis by temporarily improving
muscle function after an I.V. injection of edrophonium or neostigmine.
✔ Long-standing ocular muscle dysfunction, however, may not respond.
This test also differentiates a myasthenic crisis from a cholinergic
crisis.
✔ Edrophonium Chloride IV – 30 seconds after injection, facial muscle
weakness and ptosis should resolve for about 5 minutes
✔ Immediate improvement in muscle strength -> (+)test – confirms the
diagnosis
❖ ATROPINE SULFATE – used to control potential side effects of
edrophonium chloride => bradycardia, systole, bronchoconstriction,
sweating, cramping
2. Ice test – for px with cardiac conditions or asthma
✔ Ice pack is held over the px’s eyes for 1 minute, ptosis should
temporarily resolve
3. Electromyography (EMG)helps differentiate nerve disorders from
muscle disorders.
✔ Detects delay or failure of the neuromuscular transmission
✔ 99% sensitive in confirming dx of MS
4. Nerve conduction studies test for receptor antibodies
5. MRI – enlarged thymus gland 🡨site of acetylcholine receptor antibody
production
 MEDICAL MANAGEMENT

Goals of mgt:
✔ Improve function
✔ Reduce and remove circulating antibodies
✔ Treatment modalities include anticholinesterase medications and
immunosuppressive therapy, IVIG, therapeutic plasma exchange,
thymectomy
❑ NO CURE FOR MS; treatment does not stop the production of
acetylcholine receptor antibodies
Collaborative Mgt:
1. Myasthenic or cholinergic crisis – airway maintenance, oxygen, and mechanical
ventilation is indicated
2. Plasmapheresis - to temporarily remove circulating Ach antibodies from the blood in
crisis; to treat exacerbations
3. Thymectomy – when thymoma or hyperplasia exists; may provide remission in some
pxs
Therapeutic Mgt:
1. Anticholinergics – 1st line; enhances neuromuscular transmission; provides
symptomatic relief by inhibiting breakdown of Ach and increasing concentration of
available Ach at the neuromuscular junction
► Pyridostigmine Br -counteract fatigue and muscle weakness and enable about 80% of normal
muscle function
✔ Neostigmine – given IV in myasthenic crisis
Adverse effects:
▪ Diarrhea
▪ Abdominal cramps
▪ Excessive saliva
2. Immunomodulating drugs – reduce the production of antibody
3. Prednisone – when weakness is not controlled by anticholinergics to suppress the
immune response
✔ Initial dose given daily and maintained for 1 – 2 months
4. Cytotoxic medications – if there is an inadequate response to steroids
✔ Azathioprine – inhibits T lymphocytes and B cell proliferation; reduces acetylcholine receptor
antibody levels
Adverse effects:
▪ Leukopenia
▪ Hepatotoxicity – monthly evaluation of liver enzyme and WBC
5. IVIG – to treat exacerbations; could be used as a long-term adjunct medication
Adverse effects:
▪ HA
▪ Migraine exacerbation
▪ Aseptic meningitis
▪ Flulike sx
PROCAINE – contraindicated for patients with MG
► ATROPINE – give IV in cholinergic crisis to reduce the effects of Ach; anticholinesterase
overdose or toxicity.
PLASMAPHERESIS
❖ px’s plasma and plasma components are removed
❖ Blood cells and antibody-containing plasma are separated, then the cells and a
plasma substitute are reinfused
6. Surgery – Thymectomy
✔ Surgical removal of the thymus gland
✔ Can produce Ag-specific immunosuppression
✔ Elective surgery; done when the clinical course of the disease is stable
✔ Post-op: monitor respiratory function

Nursing intervention:
1. Monitor the patient’s respiratory rate, use of accessory muscles, and oxygen saturation to
watch for possible respiratory failure related to myasthenic or cholinergic crisis
2. Be alert for signs of impending crisis: (Myasthenic Crisis)
▪ Sudden respiratory distress
▪ Signs of dysphagia, dysarthria, ptosis, diplopia
▪ Tachycardia, anxiety
rapidly increasing weakness of extremities and trunk
3. Monitor the patient’s response to drug therapy
Supportive Care:
1. Administer medications so their peak effect coincides with meals
2. Help the patient develop a realistic activity schedule
3. Allow for rest periods throughout the day to minimize fatigue
4. If the patient has diplopia, provide an eye patch to use on the alternate eye to
minimize the risk of tripping and falling
5. Avoid aspiration
✔ teach the px to position the head in a slightly flexed position to protect the airway
during eating
✔ Have suction available that the px can operate
✔ If px is in crisis or has impaired swallowing, administer IV fluids and foods through NG
tube; elevate the head of the bed after feeding
► Plan periods of exercise, meals, patient care, and daily activities to take advantage of peaks in
the patient’s energy level.
► Provide soft, solid foods instead of liquids to reduce the risk of choking. Always sit the patient
up to eat.
► Encourage the patient to take an active role in deciding about his care.
► Evaluate the patient. Look for normal vital signs, evidence of adequate hydration and normal
elimination, skin that’s free from sores or problems, and an optimal capacity for activity.
► Encourage the patient and his family to discuss their feelings, especially feelings of frustration,
grief, or loss. Listen and provide emotional support.
► Teach the patient how to recognize adverse effects and signs of toxicity of anticholinesterase
and steroids
✔ headache
✔ weakness, sweating,
✔ abdominal cramps nausea,
✔ vomiting, diarrhea,
✔ excessive salivation, bronchospasm
► Warn him to avoid strenuous exercise, stress, infection, and unnecessary exposure to the sun or
cold weather.
► Caution him to avoid taking other medications without consulting his primary health care
provider.