0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Editorials |

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.
Text Size: A A A
Published online

Extract

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

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

First Page Preview

View Large
First page PDF preview

Figures

Tables

References

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

Related Content

Customize your page view by dragging & repositioning the boxes below.

See Also...
Articles Related By Topic
Related Collections
PubMed Articles
Jobs
brightcove.createExperiences();