Chronic Inflammatory Demyelinating Polyneuropathy

Chronic Inflammatory Demyelinating Polyneuropathy

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Chronic Inflammatory Demyelinating Polyneuropathy is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • CIDP
  • chronic relapsing polyneuropathy

Disorder Subdivisions

  • None

General Discussion

Summary

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder in which there is inflammation of nerve roots and peripheral nerves and destruction of the fatty protective covering (myelin sheath) over the nerves. This affects how fast the nerve signals are transmitted and leads to loss of nerve fibers. This causes weakness, paralysis and/or impairment in motor function, especially of the arms and legs (limbs). Sensory disturbance may also be present. The motor and sensory impairments usually affect both sides of the body (symmetrical), and the degree of severity and the course of disease may vary from case to case. Some affected individuals may follow a slow steady pattern of symptoms while others may have symptoms that stabilize and then relapse.



Introduction

CIDP is sometimes thought of as the chronic form of acute inflammatory demyelinating polyneuropathy (AIDP), the most common form of Guillain Barré syndrome (GBS), in the United States and Europe. In contrast to GBS, most patients with CIDP cannot identify a preceding viral or infectious illness. GBS is a subacute disorder that progresses over 3-4 weeks, then plateaus and usually improves over months and does not recur. CIDP, by definition has ongoing symptoms for over 8 weeks and usually does not improve unless ongoing treatment is given.

Symptoms

The chief symptoms of CIDP are slowly progressive (over at least 2 months) symmetric weakness of both muscles around the hip and shoulder as well as of the hands and feet (both proximal and distal muscles). This pattern of weakness, if caused by nerve damage, is highly suggestive of CIDP. Nerve signals become altered causing impairment in motor function and/or abnormal, or loss of, sensation. There are usually some alterations of sensation causing incoordination, numbness, tingling, or prickling sensations. Some patients only have sensory symptoms and signs but have the typical abnormalities of nerve conduction and respond to treatment as in CIDP in which weakness predominates. This is considered the sensory variant of CIDP.



Other symptoms of CIDP include fatigue, burning, pain, clumsiness, difficulty swallowing and double vision. The neurologic examination will show weak muscles that may have lost their bulk and definition (atrophy). Walking will be abnormal and responses to various sensory stimuli will be impaired.

Causes

The exact cause of CIDP is unknown but it is thought that it is related to a defect in the immune system. Although not proven, there are strong indications that CIDP is an autoimmune disorder. Autoimmune disorders occur when the body's natural defenses (antibodies and lymphocytes) against invading organisms suddenly begin to attack perfectly healthy tissue. The cause of autoimmune disorders is unknown.

Affected Populations

CIDP is a rare disorder that can affect any age group and the onset of the disorder may begin during any decade of life. CIDP affects males twice as often as females (M2:F1) and the average age of onset is 50. The prevalence of CIDP is estimated to be around 5-7 cases per 100,000 individuals.

Standard Therapies

Diagnosis

CIDP can be difficult to diagnose. The symptoms must be present for at least two months and symmetric proximal and distal weakness with reduced or absent tendon reflexes are highly suggestive of CIDP. Tests that can be of diagnostic help include nerve conduction testing and electromyography looking for very slow nerve conduction velocities, lumbar puncture looking for elevated spinal fluid protein without many inflammatory cells and MRI imaging of the nerve roots looking for enlargement and signs of inflammation.



Treatment

Glucocorticoid drugs such as prednisone have proven effective in treating individuals with CIDP. In many cases, individuals with CIDP may respond to corticosteroid treatment alone. However, individuals requiring high doses of corticosteroid drugs may experience side effects that deter long-term therapy. Corticosteroids may also be used in conjunction with other drugs such as those that suppress the immune system (immunosuppressive drugs). Azathioprine and cyclophosphamide are immunosuppressive drugs that have been used to treat CIDP.



Intravenous immunoglobulin (IVIG) has been proven to be effective and is often used as a treatment for chronic inflammatory demyelinating polyneuropathy. IVIG can enhance the immune system. Very high doses are usually used for initial treatment of CIDP and most patients require continued intermittent treatments.



Plasma exchange (PE) has also been shown to be of benefit in chronic inflammatory demyelinating polyneuropathy. This procedure is a method for removing unwanted substances (toxins, metabolic substances and plasma parts) from the blood. Blood is removed from an affected individual and blood cells are separated from plasma. The plasma is then replaced with other human plasma and the patient's blood cells are transfused back into the affected individual, thus removing only the plasma and its constituents. Similar to IVIG, PE is effective only for a few weeks and may require chronic intermittent treatments.

Investigational Therapies

There is a great deal of interest in using monoclonal antibodies to treatCIDP. Clinical trials are being developed to use rituximab, a monoclonal antibody against immune forming lymphocytes (B cells). Another monoclonal antibody under consideration is alemtuzumab which acts on both B cells and T cells, providing a broader attack on the immune system. Some of the agents that have been found to be effective in multiple sclerosis are now being considered for CIDP. A clinical trial treating patients with fingolimod, a drug that affects the ability of lymphocytes to contribute to immune function, is now underway.



Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



Contact for additional information about chronic inflammatory demyelinating polyneuropathy:



Richard A. Lewis, MD

Cedars-Sinai Medical Center

8700 Beverly Blvd.

Los Angeles, CA 90048

(310)-423-3277

ralewis49@gmail.com

References

TEXTBOOKS

Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998:625, 2464.



Adams, RD, et al., eds. Principles of Neurology. 6th ed. New York, NY: McGraw-Hill, Companies; 1997:1337-39.



Bennett JC, Plum F, eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:2152.



Menkes JH, au, Pine JW, et al., eds. Textbook of Child Neurology, 5th ed. Baltimore, MD: Williams & Wilkins; 1995:540-42.



JOURNAL ARTICLES

Dalakas MC. Advances in the diagnosis, pathogenesis and treatment of CIDP.

Nat Rev Neurol. 2011;16:507-17.



Lehmann HC, Hartung HP. Plasma exchange and intravenous immunoglobulins; mechanism of action in immune-mediated neuropathies. J Neuroimmunol. 2011;231:61-9



Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society--First Revision.

J Peripher Nerv Syst. 2010;15:1-9.



Ryan MM, et al. Childhood chronic inflammatory demyelinating polyneuropathy: clinical course and long-term outcome. Neuromuscul Disord. 2000;10:398-406.



Pou-Serradell A, Acquired dysimmune neuropathies. Clinical symptoms and classification. Rev Neurol. 2000;30:501-10.



Seoane JL, et al. Chronic demyelinating auto-immune acquired neuropathy: Lewis-Sumner syndrome. Rev Neurol. 2000;30:525-28.



Villa AM, et al. Chronic inflammatory demyelinating polyneuropathy. Findings in 30 patients. Medicina (B Aires). 1999;59:721-6.



Gorson KC, et al. Upper limb predominant, multifocal chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 1999;22:758-65.



Bouchard C, et al. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999;52:498-503.



Lewis RA. Multifocal motor neuropathy and Lewis Sumner syndrome: two distinct entities. Muscle Nerve. 1999;22:1738-39.



Nevo Y. Childhood chronic inflammatory demyelinating polyneuropathy. Europ J Paediatr Neurol. 1998;2:169-77.



Comi G, et al. Treatment of chronic inflammatory demyelinating polyneuropathy. Ital J Neurol Sci. 1998;19:261-69.



Gorson KC, et al. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997;48:321-28.



Kuwabara S, et al. Magnetic resonance imaging at the demyelinative foci in chronic inflammatory demyelinating polyneuropathy. Neurology. 1997;48:874-77.



Dyck PJ, et al. The mayo clinic experience with plasma exchange in chronic inflammatory-demyelinating polyneuropathy. Prog Clin Biol Res. 1982;106:197-204

Resources

GBS/CIDP Foundation International

The Holly Building

104 1/2 Forrest Avenue

Narberth, PA 19072

USA

Tel: (610)667-0131

Fax: (610)667-7036

Tel: (866)224-3301

Email: info@gbs-cidp.org

Internet: http://www.gbs-cidp.org/



American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



NIH/National Institute of Neurological Disorders and Stroke

P.O. Box 5801

Bethesda, MD 20824

Tel: (301)496-5751

Fax: (301)402-2186

Tel: (800)352-9424

TDD: (301)468-5981

Internet: http://www.ninds.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Guillain-Barre Syndrome Foundation of Canada, Inc.

PO Box 42016

2852 John Street

Markham, Ontario, L3R 5R0

Canada

Tel: 9056400073

Fax: 9056409815

Email: keast@sprint.ca

Internet: http://www.gbs-cidp.org or http://www.gbs-cidp.org/Canada.htm



Center for Peripheral Neuropathy

University of Chicago

5841 South Maryland Ave, MC 2030

Chicago, IL 60637

Tel: (773)702-5659

Fax: (773)702-5577

Internet: http://peripheralneuropathycenter.uchicago.edu/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



Autoimmune Information Network, Inc.

PO Box 4121

Brick, NJ 08723

Fax: (732)543-7285

Email: autoimmunehelp@aol.com



European Society for Immunodeficiencies

1-3 rue de Chantepoulet

Geneva, CH 1211

Switzerland

Tel: 410229080484

Fax: 41229069140

Email: esid@kenes.com

Internet: http://www.esid.org



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



For a Complete Report

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