MYASTHENIA GRAVIS
- Literally means "grave muscle weakness“
- An autoimmune neuromuscular disease
leading to fluctuating skeletal muscle
weakness and fatigability
- Results from failure of nerve transmission at
the neuromuscular junction due to
inadequate release of ACETYLCHOLINE or
inadequate response of muscle fibers to
acetylcholine
- Affects voluntary muscles especially those
which are innervated by the cranial nerves
3 Types of Muscles affected in MG
1. OCULAR – affects only eye and lid muscles
2. BULBAR – affects muscles for breathing,
swallowing and speaking
3. GENERALIZED – OCULAR, BULBAR + NECK and
LIMB MUSCLES (most common)
CAUSES
• Exact Cause: UNKNOWN
• Decrease in Ach secretion by the motor
end plate
• Increased acetylcholinesterase (enzyme
that destroys Ach) at the nerve endings
• Autoimmune diseases (Thymoma)
Normally:
When impulses travel down the nerve
↓
Nerve endings release a
neurotransmitter substance
↓
Ach travels through the neuromuscular junction
↓
Binds to acetylcholine receptors
↓
Activation and generation of muscle contraction
Pathophysiology
Antibodies block, alter, or destroy the receptors
for acetylcholine at the neuromuscular junction
↓
Communication between the nerve & muscle is
interrupted
↓
Muscle contraction is prevented
↓
Skeletal muscle weakness & fatigability
Clinical Manifestations:
Subjective
• Extreme muscle weakness
• Fatigue
• Dysphagia
• Diplopia- caused by weakening of
extraocular muscles
• Dysarthria
• Dyspnea
Objective:
• Ptosis
• Dysphonia- impaired ability to produce voice
• Strabismus
• Mask-like facial expression
• Myasthenic smile- SNARLING SMILE
• Drooling
• Decreasing vital capacity and respiratory
failure
DIAGNOSTIC TEST
• 1. Tensilon test
- Edrophonium chloride- a fast acting
AChE inhibitor, is administered IV to
diagnose MG.
- (+) test: immediate improvement in
muscle strength after administration of
this agent.
Snarling smile
2. - Ice pack Test
- ice is applied to the eyes for 1-2 minutes;
(+) if there’s improvement in lid strength
• 3. MRI – CHECK THYMUS GLAND
• 4. EMG – delayed/ failed nerve
– transmission
•
MEDICAL MANAGEMENT
• Directed at improving function and reducing and
removing circulating antibodies
• NO CURE: treatment does not stop production of
Ach receptor antibodies
PHARMACOLOGICAL MANAGEMENT
• Pyridostigmine Bromide (Mestinon):
anticholinesterase medication; provides
symptomatic relief
• Adverse effects: fasciculations, abdominal
pain, diarrhea, increased oropharyngeal
secretions
• Immunosuppressive drugs: to reduce the
production of the antibody
• corticosteroid (P------ ), Monitor CBC
• Azathioprine (Imuran)
• Intravenous immune globulin: used to treat
exacerbations and long- term adjunctive basis
PLASMAPHERESIS
• a technique used to treat exacerbations
• patient’s plasma and plasma components are
removed through a centrally placed large-bore
double-lumen catheter
• the blood cells & antibody containing plasma
are separated, after which the cells and a
plasma substitute are reinfused
SURGICAL MANAGEMENT
• THYMECTOMY: surgical removal of the thymus
gland; after thymus gland is removed, it may
take up to 3 years for the patient to benefit from
the procedure, because of the long life of
circulating T cells
NURSING MANAGEMENT
• Medication management- 30 mins. before meals
MEDICATIONS TO AVOID:
•Barbiturates
•Muscle relaxants
•Morphine Sulfate
•Neomycin
•Tranquilizers
• Energy conservation
- Identify the optimal time for rest throughout the
day
• Minimize the risk of aspiration
• Mealtimes should coincide with the peak effects
of anti-cholinesterase medications
• Rest before meals
• Sit upright during meals
• Soft foods- encourage gravy and sauce
• Suction should be available at home
• Supplemental feedings
• Strategies to help with ocular manifestations
- Tape the eyes closed for short intervals
- Regularly instill artificial tears
Complication
• Respiratory failure
• Impaired communication
• Corneal ulceration
• Myasthenic crisis
• Cholinergic crisis
MYASTHENIC CRISES
• Acute exacerbation of MG
• Caused by rapid, unrecognized progression of the
disease; inadequate amount of medication;
infection; fatigue; or stress
• Symptoms: respiratory distress, varying degrees
of dysphagia, dysarthria, eyelid ptosis, diplopia,
and prominent muscle weakness
MANAGEMENT:
• Patient is placed in ICU
• ET intubation and mechanical ventilation
• Provide ventilator assistance
• Ongoing assessment of respiratory failure
• Chest physical therapy
• Monitor ABG, serum electrolytes, input and
output
• NGT feeding
• Avoid sedative and tranquilizers
CHOLINERGIC CRISIS
- Results in depolarization of motor end plates
- Caused by overmedication with
anticholinesterase
Clinical Manifestations:
• Nausea and vomiting, diarrhea and abdominal
cramps
• Pallor
• Facial muscle twitching
• Hypotension
Intervention:
• Hold anticholinesterase medication
• Prepare to administer ANTIDOTE
ANTIDOTE – atropine Sulfate
(anticholinergic)
ET intubation - mechvent
