PNS PATH

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pszurnicki
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112391
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PNS PATH
Updated:
2011-10-26 18:22:58
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PNS PATH
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PNS PATH
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  1. Criteria for Myopathy?
    • muscle ache/weakness
    • CK are 10X normal
  2. 3 Infectious Polyneuropathies?
    • VZV
    • Diptheria
    • Mycobacterium Leprae
  3. What is albuminocytological Dissociation?
    WHere is it seen?
    normal cell count but elevated protein count
  4. MCC of death of NMJ and inflammatory demyelinating Neuropathy?
    respiratory failure
  5. Charcot Marie-Tooth Disease affects what ?
    • Hereditary Motor and Sensory Neuropathy
    • peroneal muscle group--atrophy..inverted bottle..thigh muscle are bulky but peroneal arent..
  6. Charcot-Marie tooth Disease affects what nervous system?
    peripheral
  7. What is deficient in Charcot-Marie-Tooth Disease?
    structural protein-connexin involved in function of peripheral nerve
  8. What 2 pathogenesis are responsible for Diabetic neuropathy?
    ischemia to nerve bc of arteriosclerosia and sorbitol accumulation casing osmotic rupture of cells
  9. How are Polymyositis and dermatomyositis different from inclusion body myositis?
    • Polymyositis and dermatomyositis involve proximal muscle groups i.e shoulder and pelvic girdle
    • Inclusion body involves distal asymmetrical myopathy
  10. What myopathy has ANA and Anti-Jo1 antibodies?
    Dermatomyositis
  11. What is the difference between Polymyositis and dermatopolymyositis?
    Both involve myopthay of proximal muscles by dermatomyositis has a rash
  12. WHich of the myopathies is associated with ca of lung, stomach, ovary?
    Dermatomyositis
  13. Periendomysial and perifascicular lymphocytic infiltrate is seen in what two myopathies?
    Polymyositis and Dermatomyositis
  14. Cytoplasmic vacuoles, eosinophilic granules and amyloid are present in what Myotpathy?
    Inclusion Body Myositis
  15. Myotonic Dystrophy presents how?
    How is it inherited?
    Whats seen on microscopy?
    How is it elicited?
    • stifness
    • CTG repeats
    • Ring fibrs
    • Percussion of thenar eminence
  16. 4 Trinucleotide repeat disease?
    • Friedrich's Ataxia
    • Mytonic dystrophy
    • Huntingtons
    • Fragile X syndrome
  17. What is the origin of Myesthenia gravis antibodies against ach receptor?
    Thymoma/THymic hyperplasia
  18. In terms of presentation how is Myesthenia gravis different from Lambert eaton syndrome?
    • MG-symptoms worsen with use
    • LES-symptoms improve with use
  19. What is the origina of Lambert eaton syndrome antibodies?
    Small cell carcinoma
  20. Diagnosis of MG?
    Tx?
    • endrophonium
    • Neostigmine, steroids, thymectomy
  21. In terms of muscles affected what is the difference between MG and LES?
    • MG--facial muscles
    • LeS-proximal--shoulder girdle
  22. How does ETOH binge drinking cause myopathy?
    rhabdomyolysis---myoglobinuria
  23. What 2 drugs can induce myopathy?
    • Steroids
    • Primaquine
  24. What endocrine disorder can cause myopathy?
    • hypo
    • hyperthyrodism
  25. What substance can cause myopathy?
    • etoh
    • steroids
    • primaquine
  26. In floopy infant with marked hypotonia, what happens to CK levels?
    unchanged
  27. Red ragged fibers are seen with what myopathies?
    Mitchondrial Neuropathy
  28. 2 conditions that have mitochondrial inheritance?
    Leiber's hereditary optic neuropathy--degeneartion of ganglion cells in the retina nd their axons
  29. Duchenes and Beckers muscular dystrophies are inherited how?
    X linked inheritance
  30. A pt using hands to get up is called what?
    Gower's sign
  31. Calf pseudohypertrophy is seen in what condition?
    Duchene's muscular dystrophy
  32. What is in the pseudohypertrophied calf muscle of Duchene kids?
    fibrous, inflammatory, adipose tissue
  33. Role of Dystrophin?
    links actin to plasma membrane
  34. What muscles are involved in Duchene's mUscular dystrophy?
    skeletal and cardiac

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