Chronic Inflammatory Demyelinating Polyneuropathy

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease that targets the myelin sheaths of peripheral nerves. Lacking a specific diagnostic test, CIDP is diagnosed on the basis of the clinical presentation and demonstration of demyelination by electrodiagnostic or nerve biopsy studies. There are many phenotype variants of CIDP. Typical CIDP involves motor and sensory nerve dysfunction, with motor deficits reported in up to 94% of patients and sensory deficits in up to 89%. Half to two-thirds of patients with CIDP display the typical phenotype. The three proven treatments for CIDP are intravenous immunoglobulin (IVIG), corticosteroids, and plasma exchange. In the last years, a set of autoantibodies against proteins located at the node of Ranvier has been identified in some patients with CIDP. IVIG treatment is not satisfactory in the majority of antibody-positive CIDP patients.