Chronic inflammatory demyelinating polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is usually idiopathic and presents with relatively symmetric, progressive or relapsing weakness, sensory loss, and hypo- or areflexia. The key to diagnosis is the presence of a sensorimotor polyneuropathy with multifocal demyelination on electrodiagnostic examination. Most patients respond well to immunotherapy. Intravenous immunoglobulin is usually considered the treatment of first choice, although a variety of other immunotherapies including plasma exchange and corticosteroids are also effective. CIDP occurs less commonly in children than in adults, but is treated similarly, and usually has an excellent prognosis. CIDP may be associated with a monoclonal gammopathy of undetermined significance. When associated with IgM, deficits are primarily sensory and response to treatment is unreliable and often poor. In contrast, when associated with IgG or IgA, deficits and treatment are similarly to those with idiopathic CIDP. An asymmetric pattern of deficits occurs and treatment is similar to idiopathic CIDP.