Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, immune-mediated polyradiculoneuritis that is progressive or relapsing over a period of at least 8 weeks. Classically, CIDP is characterized by proximal and distal weakness, large fiber sensory loss, elevated cerebrospinal fluid (CSF) protein content, demyelinating changes in nerve conduction studies or nerve biopsy, and response to immunomodulating treatment. In addition to classic sensory-motor polyneuropathy, other phenotypes of CIDP have been described. Although the exact pathogenesis is unclear, it is thought to be mediated by both cellular and humoral immune reactions directed against the peripheral nerve myelin or axon. The majority of patients with CIDP show improvement after immune therapies, including corticosteroids, plasma exchange, and high-dose intravenous immunoglobulin (IVIg).