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
- Acute/Chronic Hypoventilation (see Acute Hypoventilation, [[Acute Hypoventilation]] and Chronic Hypoventilation, [[Chronic Hypoventilation]])
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