13

Cards (21)

  • Myasthenia gravis

    Autoimmune disorder of neuromuscular junction (type II hypersensitivity)
  • Myasthenia gravis
    • Anti-acetylcholine receptor antibodies at the postsynaptic membrane of NMJ
    • Can present at any age, peaks in the third (females) and sixth (males) decade, more in women
    • Associated with thymic hyperplasia (70%), thymoma (10%) or other autoimmune disorders ex: thyroid, rheumatoid, and pernicious anemia (must screen)
  • Clinical manifestations of condition

    • Weakness and fatigability of ocular, bulbar, and proximal limb weakness
    • Weakness is descending (starts from the top and moves down)
    • Muscle fatigue after sustained/repetitive action, better with rest
    • Ocular muscles - ptosis and diplopia, better with sleep/icepack
    • Difficulty swallowing, chewing, and talking (nasal speech)
    • Respiratory difficulties
    • Diurnal variation (symptoms worse at night)
  • Bedside tests

    1. Worse when counting to 20 quickly
    2. Upward gaze - ptosis; relieved by icepacks
    3. Outstretched arms - slow downward drift
  • Normal reflexes
  • Anti-AChR antibodies

    • Most specific
  • Single fiber electromyography

    • Most sensitive
  • Nerve stimulation test

    Decrement in evoked potential
  • Anti-MuSK antibodies
    Seronegative disease; mostly ocular manifestations
  • CT
    • Thymoma/thymic hyperplasia
  • Tensilon test

    1. Giving IV anticholinesterase
    2. Temporary rapid improvement
  • Differential diagnosis of MG

    • Thyroid ophthalmopathy
    • Myotonic dystrophy
    • Brainstem cranial nerve lesions
    • Motor neuron disease
    • Lambert-Eaton myasthenic syndrome
  • Lambert-Eaton myasthenic syndrome

    • Associated with small cell lung cancer
    • Autoantibodies against presynaptic calcium channels
    • Presents with proximal muscle weakness and hyporeflexia
    • Symptoms improve with repeated muscle stimulation
  • Botulism
    • History of consumption of contaminated food (clostridium botulinum: anaerobic spore forming bacteria→neurotoxin production)
    • Acute onset of bilateral cranial neuropathies, pupillary paralysis, hyporeflexia, symmetrical descending weakness, anticholinergic symptoms
  • Anticholinesterases (pyridostigmine)
    ↑ availability of Ach at NMJ
  • Immunosuppressants
    • steroids
    • azathioprine
  • If no response/relapse on Tx
    Use immunosuppressants
  • Thymectomy
    • Indicated if thymoma is seen on CT (malignant potential)
    • Indicated if no thymoma but <50 years old & anti AchR antibodies +ve
  • Acute Myasthenic Crisis

    • Life-threatening condition
    • Profound weakness
    • Respiratory/bulbar involvement
  • Triggers for Acute Myasthenic Crisis
    • Drugs (aminoglycosides, macrolides, fluoroquinolones, phenytoin, BBs)
    • Upper respiratory tract infection
    • Surgery
    • Pregnancy/postpartum
  • Treatment for Acute Myasthenic Crisis
    1. Plasmapheresis
    2. IVIg