Chronic Inflammatory Demyelinating Polyneuropathy

Disease Overview

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) of the nerve fibers. Myelin allows nerve fibers to transmit signals very rapidly (40-60 meters/second). Loss or damage to myelin can cause slowing or blockage of the nerve signals and can lead to loss of nerve fibers. This causes weakness, paralysis and/or impairment in motor function, especially of the arms and legs. 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 person to person. 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 usually recur. CIDP, by definition has ongoing symptoms for over 8 weeks and usually does not improve unless ongoing treatment is given.

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Synonyms

• CIDP
• chronic inflammatory demyelinating polyradiculoneuropathy

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Signs & 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 a 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). Deep tendon reflexes are reduced or absent. Walking will be abnormal and responses to various sensory stimuli will be impaired.

Typical CIDP and Variants
CIDP has a number of clinical presentations. Typical CIDP is defined as a symmetric motor and sensory disorder that has proximal and distal weakness (including muscles of the shoulders and hips as well as the hands and feet). Deep tendon reflexes are absent.

Variants of CIDP include:

1. Multifocal variant in which the deficits are asymmetric and may involve different individual nerves. This form has been called Lewis-Sumner syndrome or MADSAM (multifocal acquired demyelinating sensory and motor).
2. Pure sensory variants in which patients have imbalance and incoordination but no demonstrable muscle weakness.
3. Distal variant in which the sensory and motor deficits do not involve the proximal arms and legs.
4. Pure motor variant in which no sensory abnormality is found.

The fact that there are different forms of CIDP points to the fact that at this time, CIDP is a syndrome and that there may be a number of different causes of the disorder which manifest in different ways.

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Causes

The exact cause of CIDP is unknown but 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. Recent studies have detected antibodies directed against constituents of peripheral nerve (neurofascin 155 and contactin 1) that cause rare variants of CIDP. These findings provide encouragement that the cause of other forms of CIDP will be recognized.

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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 and the average age of onset is 50. The prevalence of CIDP is estimated to be around 5-7 cases per 100,000 individuals.

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Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of CIDP. Comparisons may be useful for a differential diagnosis:

Multifocal motor neuropathy (MMN) is a rare disorder characterized by asymmetric or multifocal weakness of the arms and legs without sensory signs or symptoms. MMN usually affects one side of the body more than the other (asymmetric) and affects the arms more often than the legs. Degeneration (atrophy) of the muscles of the arms and legs is also often present. The disorder is usually slowly progressive over several years. The exact cause of MMN is unknown. (For more information on this disorder choose “multifocal motor neuropathy” as your search term in the Rare Disease Database.)

Charcot-Marie-Tooth disease (CMT) is the name given to a group of inherited neurological disorder that progressively affects movement (mobility). Peripheral nerves become enlarged or thickened causing an irregular progression of muscle weakness. Pain, weakness, numbness, and a tingling, prickling or burning sensation can occur in the legs of individuals with this disorder. Additional symptoms may include weak response of reflexes (hyporeflexia), eye abnormalities, and abnormal curvature of the spine (kyphoscoliosis). CMT frequently is noted in childhood although adult-onset forms are recognized. (For more information on this disorder choose “Charcot-Marie-Tooth disease” as your search term in the Rare Disease Database.)

Multiple sclerosis (MS) is a chronic disease of the brain and spinal cord (central nervous system) that may be progressive, relapsing and remitting, or stable. The pathology of MS consists of small lesions called plaques that may form randomly throughout the brain and spinal cord. These patches prevent proper transmission of nervous system signals and thus result in a variety of symptoms including eye abnormalities, impairment of speech, and numbness or tingling sensation in the limbs and difficulty walking. The exact cause of multiple sclerosis is unknown. (For more information on this disorder choose “multiple sclerosis” as your search term in the Rare Disease Database.)

There are a number of other peripheral neuropathies that can cause similar symptoms as CIDP. The most common neuropathy is seen with diabetes mellitus, but certain toxins, medications, alcohol and nutritional deficiencies can cause nerves to become abnormal.

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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.

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Standard Therapies

Treatment
There are a number of treatments available to control CIDP. The best studied treatments that have been shown to be effective are glucocorticoids (steroids), intravenous immunoglobulin (IVIg) and plasma exchange (PLEx). All the treatments suppress or modulate the immune system and there are increased risks of infection and cancer that must be considered when treatment decisions are being made.

Glucocorticoid drugs such as prednisone have been proven to be 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).

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. Subcutaneous delivery of immunoglobulin (SCIG) has also been shown to be effective for maintenance treatment and provides an alternative to IVIg.

Plasma exchange (PE) has also been shown to be of benefit in chronic inflammatory demyelinating polyneuropathy. This procedure is a method for removing immunoglobulins and other components of the immune response from the blood. Blood is removed from an affected individual and the blood cells are separated from plasma. The plasma is then replaced with albumin (a protein in the plasma that is not involved in immune responses) and saline (salt water) 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.

Immunosuppressive agents such as azathioprine, mycophenolate, methotrexate, cyclosporine and cyclophosphamide have been used to treat CIDP and although there are many case reports and small series pointing to efficacy, they have not been proven to be effective in large patient trials.

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Clinical Trials and Studies

There is a great deal of interest in using monoclonal antibodies (laboratory-made proteins that mimic the immune system’s ability to fight off harmful pathogens) to treat CIDP. These include rituximab, a monoclonal antibody against immune forming lymphocytes (B cells) which has been shown to be effective in treating a number of disorders including MS and rheumatoid arthritis. Treatments to reduce circulating immunoglobulins are being investigated (FcRN inhibitors) in CIDP after being shown to be effective in myasthenia gravis.

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

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

Contact for additional information about chronic inflammatory demyelinating polyneuropathy:
Richard A. Lewis, MD
Cedars-Sinai Medical Center
Dept. of Neurology
127 South San Vicente Blvd
Los Angeles, CA 90048
(310)-423-6472
Richard.lewis@cshs.org

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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

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