CIDP

Posted On March 10, 2020

Chronic inflammatory demyelinating polyneuropathy

  • Classic form:
    • symmetric, motor predominant polyneuropathy
    • both proximal and distal weakness
  • Atypical variant
    • asymmetric
    • sensory predominant
    • distal and sensory predominant
    • Proximal polyradiculopathy
    • pure motor
    • Neurofascin Ab mediated: younger, have sensory ataxia
    • Contactin 1 Ab mediated
  • Differentiate from AIDP (Guillain Barre)
    • AIDP: reaches nadir within 3-4 weeks
    • CIDP: progress or has relapses > 8 weeks
  • Other chronic demyelinating neuropathy
    • Multifocal motor neuropathy
    • Distal acquired demyelinating symmetric neuropathy: monoclonal IgM gammopathy, and anti-mag
    • POEMS syndrome: myeloma, organomegaly, endocrinopathy, monoclonal protein, skin changes
  • CIDP has been associated with hepatitis C, C, HIV, SLE, thyroid, nephrotic syndrome, solid organ or bone marrow transplant, inflammatory bowel disease.

Tests

  • Electrodiagnostic
  • Lab:
    • glucose, ha1c, Ca, creatinine
    • CBC, LFT, thyroid function
    • SPEP, UPEP
    • Other tests:
      • Lymes titer
      • CRP, ANA, angiotensin converting enzyme, hepatitis panel for B, C
      • HIV
      • Vascular endothelial growth factor if monoclonal gammopathy is found
  • LP,:
    • CSF may show protein >45, frequently over 100. WBC < 10
  • MRI cervical spine with contrast may be helpful

Diagnostic criteria

  • Progression over 2 months
  • Weakness more than sensory symptoms
  • Symmetric involvement of arms and legs
  • Proximal muscles involved with distal muscles
  • Reduced DTR
  • Increased CSF protein without elevated WBC
  • NCV showed demyelinating neuropathy

Treatment

  • Severe disease: IVIG 0.4g/kg daily for 5 days, may need to repeat q 2-6 weeks.
  • insidious: iv methylprednisolone 1 g/day x 3 days, followed by 1 g weekly x 4 weeks. slowly decrease the frequency to once every 2-12 weeks
  • failed to respond to initial treatment: a different therapy should be substituted. such as plasma exchange, and or immunosuppression with steroid.

Prognosis

  • Cure (five or more years off treatment), 11%
  • Remission (less than five years off treatment), 20%
  • Stable active disease (one or more years on treatment), 44%
  • Improving (three months to less than one year on treatment), 7%
  • Unstable active disease, 18%
Tags:,