Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Epidemiology

  • Chronic variant of Guillain-Barre Syndrome (see [[Guillian-Barre Syndrome]])
  • Unlike Guillain-Barre Syndrome, CIDP is not associated with infectious prodrome or prior immunizations

Physiology

  • Acquired demyelinating polyneuropathy (probably immune)
  • Hypoventilation leads to hypoxia/chronic respiratory acidosis

Diagnosis

  • PFT’s: restriction
  • Diaphragmatic EMG/NCV:
  • LP
    • Protein: elevated (similar to GBS)

Clinical

(CIDP is slowly progressive over months-years, with relapsing and remitting course)

  • Neuro Manifestations
    • Motor Weakness: prominent
    • Sensory/Autonomic Dysfunction: less prominent than motor symptoms
      • Parasthesias
      • Pain
  • Pulmonary Manifestations

Treatment

  • Corticosteroids: effective in subset of patients
  • IVIG: effective
    • Maintenance is recommended in responders
  • Plasmapheresis: effective, but requires long-term maintenance

Prognosis

  • Much worse than Guillain-Barre Syndrome
  • 60% of CIDP cases have a progressive course

References

  • Hahn AF, Bolton CF, Zochoden D, et al. Intravenous immunoglobulin treatment in chronic inflammatory polyneuropathy. A double blind, placebo controlled cross-over study. Brain 1996; 119:1067-1072
  • Hayn AF, Bolton CF, Pillay N, et al. Plasma exchange therapy in chronic inflammatory polyneuropathy. A double blind, sham controlled cross-over study. Brain 1996; 119:1055-1061