Chronic inflammatory demyelinating polyradiculoneuropathy

An acquired demyelinating peripheral neuropathy of presumed autoimmune etiology.

Proximal symmetric weakness is present in most patients. Atypical cases often have proximal asymmetric weakness.

Nerve conduction studies are the main diagnostic tool, but lack sensitivity; treatment should not be withheld if electrodiagnostic criteria are not met and symptoms are consistent with the diagnosis.

Treatment includes intravenous immune globulin, subcutaneous immune globulin, corticosteroids, and plasma exchange; long-term immunosuppressive therapy may be needed to prevent relapse. Lack of at least a partial response to one or two first-line agents should cast doubt on the diagnosis.

Differentiation from Guillain-Barre syndrome (GBS) is important because corticosteroids may worsen patients with GBS.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired demyelinating peripheral neuropathy of presumed autoimmune etiology. The course is usually either chronic progressive (over >8 weeks) or relapsing and remitting. The clinical phenotype consists of proximal and distal symmetric weakness, distal sensory loss, and absent reflexes.