Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated polyneuropathy characterized by symmetric weakness of proximal and distal muscles and by progression continuing > 2 mo.

Symptoms resemble those of Guillain-Barré syndrome (see Peripheral Nervous System and Motor Unit Disorders: Guillain-Barré Syndrome (GBS)). However, progression for > 2 mo differentiates CIDP from Guillain-Barré syndrome, which is monophasic and self-limited. CIDP develops in 3 to 10% of patients with Guillain-Barré syndrome.

Diagnosis

• CSF analysis and electrodiagnostic tests

Testing includes CSF analysis and electrodiagnostic tests. Results are similar to those in Guillain-Barré syndrome, including albuminocytologic dissociation (increased protein but normal WBC count) and demyelination, detected by electrodiagnostic testing. Nerve biopsy, which can also detect demyelination, is seldom needed.

Treatment

• Corticosteroids
• Sometimes plasma exchange or IV immune globulin (IVIG)

Unlike Guillain-Barré syndrome, CIDP responds to corticosteroids. However, in severe or rapidly progressive cases, plasma exchange or IVIG may be preferred. Immunosuppressants (eg, azathioprine) may be helpful and can reduce corticosteroid dependence. Treatment may be needed for a long time.

Last full review/revision August 2012 by Michael Rubin, MDCM