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Neuromuscular Junction
Neuromuscular Junction
Three basic components:
Presynaptic element Synaptic cleft Postsynaptic element
Neuromuscular Junction
Three basic components:
Presynaptic element Synaptic cleft Postsynaptic element
Plasma membrane of the axon terminal
Neuromuscular Junction
Produces:
End-plate potential
Binds to:
Acetylcholine receptors
Neuromuscular Junction
PRESYNAPTIC MEMBRANE: Axon terminal
Synaptic vesicles
Myasthenia Gravis
Grave muscle weakness Autoimmune disorder Incorporates rapid fatigue of voluntary muscle and prolonged recovery time Involves skeletal muscles
Myasthenia Gravis
TYPES:
1. Ocular Myasthenia
Males Eye muscles Good prognosis
Myasthenia Gravis
2. Generalized Myasthenia
Proximal musculature Periodic remissions, slowly or rapidly progressive course or a fulminating course
3. Bulbar Myasthenia
Cranial nerves IX, X, XI and XII Rapidly progressive or fulminating
Myasthenia Gravis
Other Types:
Transient Neonatal Myasthenia
Cause: Antibodies produced by the mother that crosses the placental barrier Lasts a few days or weeks and does not recur
Myasthenia Syndrome
Associated with neoplasms Similar manifestations as with MG Results from decrease in release of ACh from nerve terminals, not destruction of ACh receptors
Myasthenia Gravis
HALLMARKS:
Fluctuating weakness of voluntary muscles: facial and extraocular Muscle weakness that increases with activity Muscle strength that improves with rest Muscle strength that improves in response to anticholinesterase medications
ACh and chemical stimuli binds to ACh receptor site on the post synaptic membrane
Depolarization
Muscle contraction
Myasthenia Gravis
Pathophysiology:
Pathologic thymus changes Age and Gender
F 20 M 70-80 Placental transfer of ACh receptor antibodies
Autoimmune
SLE, RhA
Mothers with MG
Generation of autoantibodies
Ocular
Facial
Peripheral
Respi
Myasthenia Gravis
Manifestations:
Ocular
Peripheral
Weakness Fatigue
Myasthenia Gravis
Manifestations:
Facial Expressionless face Facial droop Snarling appearance Hanging jaw sign
Respiratory
Impaired gag and cough reflex Inability to expel secretions Respiratory distress
Diagnostic Tests
Physical Examination Medical History Immunoassay: ACh Receptor antibody Nerve stimulation studies MRI: Thymoma Tensilon test
Edrophonium chloride 25g 30 seconds (+) Improved strength for a few minutes (-) No improvement or gets worse
Brittle Myasthenics:
Management
MEDICATIONS Anticholinesterase (Cholinergic)
Neostigmine (Prostigmine) Pyridostigmine (Mestinon)
Available in time span form and is often used at bedtime
Management
MEDICATIONS Corticosteroids
Reduce antibody production
Immunosupressants
Dont respond to steroids NR: prevent infection
Discharge Instructions
Signs and symptoms of exacerbation Avoid overexertion and allow time for rest Avoid mycin antibiotics and meds containing quinine Avoid alcohol
Morphine and meperidine depresses respiratory function and must be avoided Adequate nutrition: increase resistance, decrease infection Schedule of medication Wearing of tag
Guillain-Barr Syndrome
Acute demyelinating polyneuropathy
Involves demyelination or axonal degeneration of multiple peripheral nerves
Other names:
Infectious polyneuritis Idiopathic polyneuritis Landrys ascending paralysis Acute inflammatory polyneuropathy
MEDICAL EMERGENCY!
Sequence of events:
1. Precipitating event 2. Changes the cells in the nervous system 3. Immune system recognizes cells as nonself or foreign 4. Sensitized T lymphocytes ad macrophages attack myelin 5. T lymphocytes stimulate B lymphocytes to produce antibodies against myelin sheaths
RESULT: Demyelinating injury that interferes with impulse conduction in the affected peripheral nerve If cell body is not destroyed, peripheral nerve regeneration may take place If cell body dies, nerve regeneration cant take place
Decreased sensation
Sensory fiber damage
Assessment
Skeletal muscle Loss of muscle tone Areflexia Tenderness
Upon deep pressure
Assessment
Cranial Nerves Facial palsy Speech difficulties Visual disturbances Swallowing difficulties Bulbar palsy
Paralysis of the jaw, pharynx and tongue
Diagnostic Tests
1. EMG 2. Nerve conduction velocity 3. Lumbar puncture LUMBAR PUNCTURE High protein levels in CSF later in the course
Active demyelination
Management
Supportive
Pain, ventilation, blood pressure and cardiac function
Plasmapheresis Immunoglobulins
Intensive physiotherapy
As soon as voluntary movement returns to prevent muscle and joint contractures
Rehabilitation
RESPIRATORY STATUS Ensure oxygenation via ventilatory assistance Prevent immobility complications Tracheostomy