Chronic Inflammatory Demyelinating Polyradiculoneuropathy

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Chronic Inflammatory Demyelinating Polyradiculoneuropathy

(CIDP)

  • slowly progressive, sometimes relapsing steroid-dependent demyelinating polyneuropathy primarily affecting limbs

Presentation

  • any age, slightly increased incidence in males

  • Chronic relapsing or chronic progressive course

    • greater than 8 weeks to maximal distinguishes from Guillain-Barré syndrome

    • 50% have relapsing / remitting course that may progress to steady downhill decline

  • 3/4 patients have mixed symmetrical sensorimotor loss

  • paraesthesia

  • limb weakness both proximal and distal

Signs

  • loss of vibration and joint position sense

  • reflexes reduced or absent

  • cranila nerves involved in ~15%

  • papilloedema present in small proportion

  • coarse tremor

Investigations

  • CSF

    • increased protein (>1g/l)

  • Nerve conduction studies

    • definitive - slowed nerve conduction in chronically / subacute progressive acquired peripheral neuropathy

Aetiology / Pathology

  • 1/3 have preceding viral infection or vaccination
    • ? relation to CMV
  • widespread demyelination
  • stripping of myelin by macrophages
  • intrafascicular oedema
  • hypertrophic changes
  • associated CNS involvement
    • periventricular plagues
    • may resemble multiple sclerosis

Differential diagnoses

  • Guillain-Barré syndrome
    • GBS has more acute onset, not treated with steroids
  • Hereditary Motor and Sensory Neuropathy
    • HMSN tends to involve fascicles uniformly

Treatment

  • oral steroids may give improvement by 3 weeks
    • 60mg/ day for 2 weeks
    • 45 mg / day progressing to 45 mg/ other day over 2-3 months
    • may be needed for years
    • often relapse a few months after stopping
  • azathioprine
    • tried for steroid sparing activity but no good evidence
  • cyclosporin A improvement in some steroid resistant cases
  • plasma exchange - gives a few weeks improvement
  • Iv - immunoglobulin
    • 0.4 mg/kg/day over 3-5 days

Prognosis

  • fatal in 10% in untreated
  • Excellent response to steroid therapy
  • Elderly / significant axonal involvement do badly
 

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