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M S . S H A M A P R A V E E N
T U T O R P H Y S I O L O G Y
Neuromuscular dysfunction
Myasthenia Gravis
 Autoimmune disease
 Characterized by weakness and fatigability of skeletal
muscles that gradually worsens as the muscles are
used.
 Decrease in the number of AChR present on the
motor end plate due to production of circulating
autoantibodies against these receptors.
 Postsynaptic folds are flattened or simplified.
Features
 Due to postsynaptic changes along with the presynaptic
rundown, after a few motor nerve impulses, the
successive contractile responses become feebler, causing
fatigue.
 Women are affected more than men in a ratio of 3:2.
 The muscle weakness increases during prolonged use of
the muscle & improves after rest or sleep.
 The extraocular muscles and eyelids are often involved
early in the course of diseases. Therefore, diplopia and
ptosis (drooping of eyelids) are early symptoms.
 Proximal limb muscles are commonly affected. In severe
cases, respiratory muscle paralysis leads to death.
Ptosis seen in Myasthenia Gravis
Physiological basis of treatment
 Administration of AChE inhibitors: Pyridostigmine
& neostigmine (increase the concentration of Ach at
the neuromuscular junction.
 Thymectomy: it blunts down the immune response
and improve the condition.
 Immunosuppression: immunosuppressive drugs
glucocoticoids and azathioprine are useful.
 Plasmapheresis: it removes AChR antibodies from
plasma.
Lambert- Eaton Myasthenic Syndrome
 Presynaptic disorder of the neuromuscular junction due
to production of autoantibodies against voltage- gated
Ca++ channels.
 The decreased Ca++ influx in the presynaptic axon
terminals results in impaired Ach release from the nerve
endings.
 The muscular weakness is primarily seen in the limb
muscles.
 Patients show incremental response to repetitive nerve
stimulation as calcium level raises with AP. With
prolonged contractions, muscle strength increases.
THANK YOU

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Neuromuscular dysfunction.pptx

  • 1. M S . S H A M A P R A V E E N T U T O R P H Y S I O L O G Y Neuromuscular dysfunction
  • 2. Myasthenia Gravis  Autoimmune disease  Characterized by weakness and fatigability of skeletal muscles that gradually worsens as the muscles are used.  Decrease in the number of AChR present on the motor end plate due to production of circulating autoantibodies against these receptors.  Postsynaptic folds are flattened or simplified.
  • 3. Features  Due to postsynaptic changes along with the presynaptic rundown, after a few motor nerve impulses, the successive contractile responses become feebler, causing fatigue.  Women are affected more than men in a ratio of 3:2.  The muscle weakness increases during prolonged use of the muscle & improves after rest or sleep.  The extraocular muscles and eyelids are often involved early in the course of diseases. Therefore, diplopia and ptosis (drooping of eyelids) are early symptoms.  Proximal limb muscles are commonly affected. In severe cases, respiratory muscle paralysis leads to death.
  • 4. Ptosis seen in Myasthenia Gravis
  • 5. Physiological basis of treatment  Administration of AChE inhibitors: Pyridostigmine & neostigmine (increase the concentration of Ach at the neuromuscular junction.  Thymectomy: it blunts down the immune response and improve the condition.  Immunosuppression: immunosuppressive drugs glucocoticoids and azathioprine are useful.  Plasmapheresis: it removes AChR antibodies from plasma.
  • 6. Lambert- Eaton Myasthenic Syndrome  Presynaptic disorder of the neuromuscular junction due to production of autoantibodies against voltage- gated Ca++ channels.  The decreased Ca++ influx in the presynaptic axon terminals results in impaired Ach release from the nerve endings.  The muscular weakness is primarily seen in the limb muscles.  Patients show incremental response to repetitive nerve stimulation as calcium level raises with AP. With prolonged contractions, muscle strength increases.
  • 7.