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Improvement of Axonal Function in Patients With Chronic Inflammatory Demyelinating Polyneuropathy After Intravenous Immunoglobulin Therapy

Richard A. C. Hughes, MD, FRCP; Kerry Mills, PhD
Arch Neurol. 2011;68(7):844-846. doi:10.1001/archneurol.2011.138.
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Since the first clear descriptions by Austin in 1958,1 chronic inflammatory demyelinating polyneuropathy (CIDP) has emerged as a definable, not uncommon, and treatable cause of chronic peripheral nerve disease. After many different individual definitions and classifications, an international consensus group set diagnostic criteria that identify definite and probable cases, depending on the severity of the neurophysiological abnormalities, and that distinguish typical symmetrical cases with sensory and motor involvement and predominantly proximal weakness from various atypical cases with asymmetrical, pure motor, pure sensory, or other features.2 Recent epidemiological studies and series suggest a prevalence for CIDP of up to 9 per 100 000 population, with the highest figure emerging from regions with the most sophisticated neurological diagnostic services.3 The severity of the disease is variable, and although some patients have little or no disability, most have significant symptoms, often including fatigue; in our recent southeast England prevalence study,4 over 30% were so disabled that they required assistance to walk. Most patients require treatment. Use of corticosteroids, plasma exchange, and intravenous immunoglobulin (IVIG) have all been shown in randomized controlled trials to provide short-term benefit, and 1 brand of IVIG has been shown to produce long-term benefit when given every 3 weeks for 24 weeks.5 Although nerve biopsies have limited diagnostic value, fortuitous biopsies of active lesions have shown macrophage-associated demyelination, demyelinated and remyelinated axons, and onion bulbs indicating recurrent episodes of demyelination and remyelination. These histological appearances have been mimicked by chronic experimental models in which rats have been immunized with myelin and adjuvant. More recently, chronic inflammatory neuropathy has been shown to develop spontaneously in nonobese diabetic mice genetically modified to lack molecules that regulate T cells.6 One important antigen in these models is the major myelin glycoprotein P0.6

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