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Neurology Chapter of IAP
Myasthenia Gravis
• Disease of the neuromuscular junction
characterized by fluctuating weakness
of certain skeletal muscle groups.
Neurology Chapter of IAP
Myasthenia Gravis(MG)
• Acetycholine (ACh) is an important
neurotransmitter that stimulates muscle
tissue to contract.
• MG is an autoimmune disease in which
antibodies are formed against ACh and
a reduction in ACh receptor sites at the
neuromuscular junction.
Neurology Chapter of IAP
•
Neurology Chapter of IAP
Pathophysiology
• Loss of muscle strength.
• There is no single cause identified,
however, thymic tumors and viral
infections have been found in a certain
number of patients.
Neurology Chapter of IAP
Clinical manifestations
• Primary s/s= easy fatigability of skeletal
muscle during activity.
• Muscles involved: eyes and eyelids,
chewing, swallowing, speaking, and
breathing.
• Fluctuating weakness: usually strong in
the a.m., progressively weaker with
activity.
Neurology Chapter of IAP
Clinical Manifestations
• 90% of patients have eye involvement
• Facial mobility may be impaired
• Muscles of limb and trunk less often
affected.
• No sensory or reflex loss; muscle
atrophy is rare.
Neurology Chapter of IAP
Clinical manifestations
• Variable course
• May be precipitated by emotional
stress, pregnancy, menses, secondary
illness, trauma, temperature extremes,
hypokalemia, ingestion of drugs with
neuromuscular blocking agents,
surgery.
Neurology Chapter of IAP
Complications
• Aspiration, respiratory insufficiency, and
respiratory infection
• Acute exacerbation called myasthenic
crisis.
• The opposite of this is a cholinergic
crisis and results from overdose of
cholinergic drugs.
Neurology Chapter of IAP
Diagnostic studies
• Assessment:
– Have pt look up for 2-3 minutes; if MG, patient will have
increased droop of eyelids.
– EMG may show muscle fatigue
– Tensilon test- in MG reveal improved muscle contractility
after IV anticholinesterase agent edrophonium chloride
(tensilon)
– Also diagnosis cholinergic crisis- muscle weakness gets
worse
– Keep atropine on hand to counteract effects of tensilon
Neurology Chapter of IAP
Therapeutic management
• Anticholinesterase inhibitors- prevents
anticholinestersase from breaking down ACh;
helps neurotransmission. Monitor dose!
– Mestinon, Prostigmine
Corticosteroids- decrease immune response
Prednisone
Plasmapheresis- removes ACh antibodies
and short-term improvement.

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Myasthenia Gravis Pathophysiology and Treatment

  • 1. Neurology Chapter of IAP Myasthenia Gravis • Disease of the neuromuscular junction characterized by fluctuating weakness of certain skeletal muscle groups.
  • 2. Neurology Chapter of IAP Myasthenia Gravis(MG) • Acetycholine (ACh) is an important neurotransmitter that stimulates muscle tissue to contract. • MG is an autoimmune disease in which antibodies are formed against ACh and a reduction in ACh receptor sites at the neuromuscular junction.
  • 4. Neurology Chapter of IAP Pathophysiology • Loss of muscle strength. • There is no single cause identified, however, thymic tumors and viral infections have been found in a certain number of patients.
  • 5. Neurology Chapter of IAP Clinical manifestations • Primary s/s= easy fatigability of skeletal muscle during activity. • Muscles involved: eyes and eyelids, chewing, swallowing, speaking, and breathing. • Fluctuating weakness: usually strong in the a.m., progressively weaker with activity.
  • 6. Neurology Chapter of IAP Clinical Manifestations • 90% of patients have eye involvement • Facial mobility may be impaired • Muscles of limb and trunk less often affected. • No sensory or reflex loss; muscle atrophy is rare.
  • 7. Neurology Chapter of IAP Clinical manifestations • Variable course • May be precipitated by emotional stress, pregnancy, menses, secondary illness, trauma, temperature extremes, hypokalemia, ingestion of drugs with neuromuscular blocking agents, surgery.
  • 8. Neurology Chapter of IAP Complications • Aspiration, respiratory insufficiency, and respiratory infection • Acute exacerbation called myasthenic crisis. • The opposite of this is a cholinergic crisis and results from overdose of cholinergic drugs.
  • 9. Neurology Chapter of IAP Diagnostic studies • Assessment: – Have pt look up for 2-3 minutes; if MG, patient will have increased droop of eyelids. – EMG may show muscle fatigue – Tensilon test- in MG reveal improved muscle contractility after IV anticholinesterase agent edrophonium chloride (tensilon) – Also diagnosis cholinergic crisis- muscle weakness gets worse – Keep atropine on hand to counteract effects of tensilon
  • 10. Neurology Chapter of IAP Therapeutic management • Anticholinesterase inhibitors- prevents anticholinestersase from breaking down ACh; helps neurotransmission. Monitor dose! – Mestinon, Prostigmine Corticosteroids- decrease immune response Prednisone Plasmapheresis- removes ACh antibodies and short-term improvement.