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, azathioprineSome Trade Names
IMURAN
Click for Drug Monograph
) 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

Content last modified August 2012