CIDP 5

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Author:
xijunzhu
ID:
310925
Filename:
CIDP 5
Updated:
2015-11-04 20:39:13
Tags:
cidp
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Description:
exam 2
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  1. trophic changes
    • due to interruption of nerve supply
    • pes cavus
    • kyphoscoliosis
    • loss of hair
    • charcot joints (neurogenic arthropathy)
  2. sensation that suggests neuropathy
    • vibration/proprioception (large myelinated axon)
    • temp/pain (small myelinated axon)
  3. chronic
    over one month
  4. why is it a polyneuropathy?
    • motor and sensory
    • symmetrical (or more than one limb)
    • non-length dependent
  5. what percentage of patients had class triad?
    30% in patients who all had proximal and distal weakness and in whom 95% had improvement with treatment
  6. classic triad
    • slow nerve conduction velocity
    • elevated CSF protein
    • demyelination on nerve biopsy
  7. american academy of neurology criteria says must have three of four
    • conduction block
    • decreased conduction velocity
    • increased distal latency
    • increased F-wave latency
  8. what characteristics in CSF support CIDP
    • WBC<10
    • VDRL neg
    • elevated protein
    • criteria by aanc is not sensitive and may miss more than 50% of the patients
  9. protein level is increased in how many patients?
    80%
  10. demyelinating neuropathy
    • multifocal conduction block or temporal dispersion of CMAP
    • prolonged distal latencies
    • variable conduction slowing to less than 70% normal
    • absent or prolonged F wave latencies
  11. histological findings
    • interstitial and perivascular infiltration of endoneurium with T cells and macrophages
    • segmental demyelation and remyelation (onion bulb)
    • neutrophil infiltration
    • axonal damage/loss of myelinated nerve fibers
  12. histology on picture of CIDP
    • decreased density of nerve fibers
    • demyelinated fibers
    • fibers undergoing active macrophage mediated demyelination
  13. what is the prevalence of the disease?
    1-7.7 per 100,000
  14. pathophysiology (the disordered physiological process associated with the disease)
    • gradual onset (difficult to document)
    • respiratory and gastrointestinal infections have been cited, no causative organism identified
    • GBS (Campylobacter) most common preceding infection
  15. IgM
    produced by B cells is the first antibody to appear in response to antigen
  16. phenotype of CIDP
    • proximal and distal weakness
    • large fiber sensory loss and areflexia
  17. MS
    CNS white matter changes in patients with CIDP
  18. CIDP associated conditions
    • diabetes mellitus predispose patients to CIDP
    • pregnancy (3rd trimester or postpartum)
  19. goal and course of treatment
    • goal: block immune process
    • course: continue until maximal improvement then maintain
  20. treatments
    • immunosuppressants
    • interfereons 
    • decoy molecules
    • t cell vaccination (destroy population of autoimmune T cells)
  21. how many percentage of patients have sustained remission after initial treatment
    1/3
  22. early treatment why?
    prevent axonal and motor neuron loss
  23. what is the first line therapy
    prednisone
  24. agents used for refractory patients?
    • cyclosporin 
    • cyclophosphamide
    • azathioprine
    • mycophenolate
  25. anti-epileptics
    carbamazepine
  26. tricyclic antidepressants
    elavil
  27. common treatment regime for plasma exchanges
    3 per week for first 2 weeks
  28. IVIg
    composed of heterogeneous human IgG and smaller amounts of IgA and IgM
  29. mechanism of IVIg
    • random set of antibodies to neutralize immune factors causing damage to peripheral nerve
    • activate complement cascades and antibodies against toxins, etc
    • provide immediate passive immunity
    • seen at day 10 continues to day 42
  30. serum half life
    • 21-29 days
    • patients require repeated treatments to maintain remission or treat recurrences

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