myasthenia gravis Flashcards
major issue with myasthenia gravis
not enough acetocholine
myasthenia gravis definition
Definition:
a. Disease of the neuromuscular junction characterized by fluctuating weakness of certain skeletal muscle groups especially the face & throat. b. Affects neuromuscular transmission of voluntary muscles of the body especially those innervated by the cranial nerves.
issues with myasthenia gravis
c. Is purely a motor disorder with no effect on sensation or coordination.
d. Results in inability for muscles to contract.
e. There are periods of remission & exacerbations and a milder form does exist.
f. Considered an autoimmune disease where antibodies destroy or block neuromuscular acetylcholine receptor sites.
severity of myasthenia gravis
there are many different levels of this disease from mild symptoms to debilitating
thymus gland and myasthenia gravis
. Thymus gland, which is usually inactive after puberty, continues to produce antibodies. It is believed that the thymus is the source of autoantigen that triggers the autoimmune response inMG.
H. Prevents acetycholine from attaching to receptor site and from stimulating muscle contraction.
viral infections and myasthenia gravis
Viral infections are suspected of precipitating an attack.
statistics on myasthenia gravis
43 to 84 persons/ million affected.
Peak age of onset in women is 20 to 40 yrs. Majority of men are over 50 yrs.
Women are affected 3X more than men
manifestations of myasthenia gravis
Fatigue and weakness of skeletal muscles generally worse with effort and relieved by rest. Patients can tire from combing hair or chewing.
bulbar symptoms of myasthenia gravis
Bulbar symptoms:
a. Weakness of face & throat
b. Diplopia ( eye droop) , Ptosis, facial weakness, dysarthria, dysphagia, dysphonia.
Complications: Difficulty closing eyes, aspiration, impaired communication & nutrition
musculoskeletal and respiratory problems in myasthenia gravis
decreased function of hands, arms, legs and neck muscles.
b. Weakening of intercostal muscles , decreased diaphragm movement, dyspnea, and poor gas exchange.
Complications:
a. Decreased ability to perform ADLs
b. Immobility complications
c. Inability to walk or eat
d. Respiratory infections.
There is no sensory loss. Reflexes are normal and muscle atrophy is rare.
the course of myasthenia gravis
The course of the disease varies. Some have short-term remissions, others stabilize and others have severe progressive involvement.
exacerbations of myasthenia gravis
Exacerbations can be precipitated by emotional stress, pregnancy, menses, illness, trauma, temperature extremes, hypokalemia, surgery & drugs
diagnosis of myasthenia gravis
Patient history and physical exam
Have person look upward for 2 to 3 minutes. If MG there will be an increased droop of the eyelids so the person can barely keep eyes open.
IV tensilon (anticholinesterase) test
Electromyography ( EMG ) to measure electrical potential of muscle cells
Anti-ACH receptor antibodies in serum. Seen in 70 to 80 % of patients.
IV tensilon (anticholinesterase) test
a. Drug facilitates transmission of impulses at myoneural junction.
b. Within 30 seconds of injection most patients with MG improve substantially but is only temporary.
prognosis of myasthenia gravis
No known cure but drug therapy helps
medical management of myasthenia gravis
Drug Therapy: Maintenance of stable blood levels of medications is imperative to stabilize muscle strength. Meds must be given on time.
a. Anticholinesterase Inhibitors:
1. Mestinon ( Pyridostigmine Bromide )
2. Prostigmin ( Neostigmin Bromide
b. Corticosteroids
c. Immunosuppressive Therapy
Plasmapheresis
Surgical Management: Thymectomy
a. Results in symptom remission in about
40%. Best if within 2 years of diagnosis
what to remember about myasthenia gravis
drugs need to be on time. Do not want to be late. need to maintain a constant blood level
anticholinesterase inhibitors for Myasthenia gravis
. Drugs increase available acetycholine at the junction. Given to increase the response of the muscles to nerve impulses to improve strength. Gives symptomatic relief. The maintenance dose is determined by testing.
B. Maintenance of stable blood levels of these meds is imperative to stabilize muscle strength. Meds must be given on time since a delay can exacerbate muscle weakness and make it impossible for the patient to take meds by mouth.
C. Common side effects of these drugs:
CNS—irritability, anxiety, insomnia, headache, dysarthria, syncope, seizures,
coma, diaphoresis
RESP—increased bronchial secretions, bronchial spasm
CARDIO—hypotension, tachycardia
GI—abdominal cramps, nausea, vomiting, diarrhea, anorexia, increase salivation
SKELETAL—spasms, fine twitching, weakness of muscles
INTEG—rash, flushing, increased lacrimation
D. Examples:
1. PYRIDOSTIGMINE BROMIDE (MESTINON)
a. Most commonly used. Peaks in2hours. Duration is 3-6 hours
2. PROSTIGMIN (NEOSTIGMINE BROMIDE)
a. Has less side effects than Mestinon. Dosage is increased gradually until
maximum benefits.
b. Give with milk, crackers, and buffering substances for GI distress. Small
doses of Atropine may be given to decrease above side effects.
corticosteroids and immunosuppresive therapy for myasthenia gravis
II. CORTICOSTEROIDS:
A. Suppress immune response and decrease amount of antibody production.
B. May use alternate day therapy.
III. IMMUNOSUPPRESSIVE THERAPY:
A. Reduces production of antireceptor antibodies. Are cytotoxic meds.
B. Use drugs like Imuran, Cytoxan.
common side effects of anti cholinesterase drugs
CNS: Irritability, fine twitching, anxiety, insomnia, headache, dysarthria, syncope, seizures, coma, spasms, weakness
RESP: Bronchial spasm, increased bronchial secretions
Cardio: Hypotension, bradycardia
GI: Abdominal cramps, nausea, vomiting, diarrhea, heartburn, anorexia, increased salivation
Skin: rash, flushing, sweating, tearing
***Myasthenia Crisis
Usually caused by under medication. Can be triggered by infection, surgery, emotional stressors, and drugs. Many drugs are contraindicated or must be used with caution. These include anesthetics, antidysrhythmics, antibiotics, quinine, hypnotics, cathartics, diuretics, antipsychotics, barbituates, muscle relaxants, thyroid medications, sedatives, tranquilizers
how is a myasthenia crisis manifested
Manifested by acute exacerbation of muscular weakness with acute respiratory distress, and inability to swallow or speak.
Weakness of respiratory muscles causes respiratory depression and airway obstruction.
Can have periods of apnea, extreme fatigue, fever, anxiety, restlessness, irritability, unable to move jaw and raise one or both eyelids.
how can you determine its a myasthenia crisis
Need to confirm with Tensilon Test
May need plasmaphresis. Given meds parenterally until able to swallow
Will be in ICU. Considered medical emergency.
cholinergic crisis
Over medication is usually the cause.
In addition to muscle weakness and respiratory depression, patients will have a variety of GI symptoms.
Is a medical emergency. Will be in ICU. Confirm with Tensilon Test.