Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic, acquired immune and inflammatory disorder of the peripheral nervous system. The classic form of the disorder is manifested by progressive or relapsing proximal or generalized limb weakness and areflexia, and usually easily recognized; it is the large number of regional and functional variants and variety of associated illnesses that pose a challenge to the clinician in practice. Similarly, laboratory and electromyography criteria have been developed to confirm the diagnosis; however, these various schemes are contrived because only 50% to 60% of patients with typical clinical features of CIDP fulfill these strict electrodiagnostic research criteria. Several studies have established the efficacy of immune therapies such as corticosteroids, plasma exchange, and intravenous immune globulin as the mainstay of treatment of CIDP, but these treatments might provide only short-term benefit. This review offers an approach to the evaluation and management of patients with CIDP and highlights the difficult clinical problems in those who do not respond or frequently relapse after treatment with standard therapies such as patients with CIDP and concomitant axonal loss, and the assessment of those with CIDP and concurrent diseases such as diabetes mellitus.