Chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

For much of the 20th century, our understanding of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) was limited because the condition went by several different names; clinical and histopathologic descriptions were often combined with those of similar neuropathic disorders including Guillain-Barré syndrome, the hereditary hypertrophic polyneuropathies, and other chronic polyneuropathies; and the means to sufficiently study and treat the condition had not been developed. Major advances in the understanding of CIDP occurred mid century and included the development of the experimental allergic neuritis (EAN) model, the elaboration of nerve conduction studies, and the discovery of corticosteroids. In the 1970s, CIDP finally emerged as a distinct entity with well-defined clinical, histopathologic, and electrodiagnostic features. Diagnostic criteria were developed, and subsequent treatment trials proved the efficacy of corticosteroids, plasma exchange, and intravenous immunoglobulin.