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Favopur Agabi
Ryan Millikan
Roxana Lupu
· Myasthenia gravis is an autoimmune neurological disease characterized by impaired communication between nerves and muscles at the neuromuscular junction.
· It is an antibody-mediated disease or a class II hypersensitivity reaction.
· The antibodies, produced by the thymus gland and blood cells, prevent the neurotransmitter acetylcholine to bind to nicotinic acetylcholine neuro receptors on the muscle membrane or they can destroy these neuroreceptors. Thus, the influx of sodium into the muscles is blocked resulting in the impossibility of depolarization of the muscle. Therefore, the transmission of nerve impulses is blocked and the muscles fail to contract properly.
· The outcome is a fluctuating muscle weakness that is getting worse in the second part of the day.
Myasthenia gravis is caused by a loss of acetylcholine receptor in the post synaptic neurons at the neuromuscular junction.
- About 80% of all MG patients have elevated titer for ACHr antibodies which can prevent the acetylcholine molecule from binding to these receptors sites or can cause damage to them
Primary risk factor is heredity
In the age range of 20-30, women are more likely to be affected than men. However by the age of 50, more men are affected
Date
Pyridostigmine
- It is an Indirect-Acting Cholinergic Agonist, a reversible cholinesterase inhibitor that increases the levels of acetylcholine facilitating transmission at the neuromuscular junction.
- It can be given PO, IM, or IV.
- Adverse effects: Bradycardia, cardiac arrest, salivation, tearing, nausea, dysphagia, vomiting, increased bronchial secretions, urinary frequency, and incontinence.
Characteristic findings
o Weakness and fatiguability (in certain muscles
such as extraocular, bulbar, limb muscles)
o The weakness and fatiguability is improved by
rest of anticholinesterase medication.
- Stops cholinesterase from breaking
down acetylcholine in the synaptic cleft
o Electromyography (EMG): decrease in
compound muscle action potential (CMAP)
amplitude during repetitive motor nerve
stimulation
- Chest CT or MRI may reveal thymic enlargement or mass (thymus hyperplasia)
- Presence of the AChR antibodies
· Ensure a patent airway.
· Monitor heart functions.
· Have emergency respiratory equipment readily available.
· Provide psychological and emotional support
· Provide frequent rest periods.
· Assess muscle strength, functioning, and fatigability.
· Turn and reposition the patient regularly and frequently.
· Provide skin care.
· Apply antiembolism stockings or sequential compression stockings to prevent VTE.
· Maintain a nutritional management program. Encourage the use of thickened liquids if the patient has difficulty swallowing.
· Encourage socialization.
Occular Bulbar
-Diplopia (double vision) -Dysarthria
-Ptosis (drooping eye) -Dysphagia
-Eye muscle weakness
Respiratory Neck/Limbs
-Shortness of breath -Dropped head
-Weakness in
arms/legs/fingers/hands
Complication
- Acute Exacerbation called myasthenic crisis (life threatening muscle weakness of diaphragm and chest muscle)
- Repiratory Failure- breathing is shallow and ineffective (can lead to death if not controlled)
- People diagnosed with this disease have a higher risk of getting other autoimmune disorders like lupus and rheumatoid arthritis