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NORD is very grateful to Richard A. Lewis, MD, Cedars-Sinai Medical Center, for assistance in the preparation of this report.
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.
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.
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.
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.
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.
Symptoms of the following disorders can be similar to those of chronic inflammatory demyelinating polyneuropathy. Comparisons may be useful for a differential diagnosis:
Lewis-Sumner syndrome is a rare neurological disorder characterized by asymmetric or multifocal weakness and sensory dysfunction affecting the arms and legs (limbs). In some cases, weak responses of the tendon reflexes may be present. Some researchers believe that Lewis-Sumner syndrome is a variant of CIDP. Others believe it, along with multifocal motor neuropathy, represents part of a disease spectrum. The exact cause of Lewis-Sumner syndrome is unknown.
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 (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.
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.
Multiple sclerosis 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.
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.
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.
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
For information about clinical trials sponsored by private sources, contact:
Contact for additional information about chronic inflammatory demyelinating polyneuropathy:
Richard A. Lewis, MD
Cedars-Sinai Medical Center
8700 Beverly Blvd.
Los Angeles, CA 90048
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Report last updated: 2012/10/26 00:00:00 GMT+0