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What Is Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?

In this Article

In this Article

In this Article

  • What causes it?
  • Is it the same as Guillain-Barre syndrome?
  • How is it diagnosed?
  • What do you do to feel better?

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder — a condition that targets your body’s nerves.

Symptoms aren’t the same for everyone, but you may be tired and have areas of numbness and pain. It can slow your reflexes and make your arms and legs feel weak. You have to have symptoms for at least 8 weeks for CIDP to be considered the cause.

Most people need treatment. And the sooner you begin it, the better the chance of a complete recovery. Sometimes symptoms go away for a long time but come back later.

Anyone can get CIDP, but it’s most common in older adults, and more in men than women. As many as 40,000 people in the U.S. may have the condition, but it’s hard to know how many people have it. CIDP isn’t easy to diagnose.

What causes it?

Experts aren’t sure why people get the disorder. What they do know is that it’s caused by inflammation of nerves and nerve roots. The swelling can destroy the protective covering around nerves, known as myelin . That can hurt nerve fibers and slow the nerves’ ability to send signals. This is what causes the weakness, pain , fatigue , and numbness.

Is it the same as Guillain-Barre syndrome?

No. CIDP is closely related to Guillain -Barre syndrome (GBS). Both are nerve problems, and both cause symptoms such as weakness and numbness. But GBS usually comes on days or weeks after a person has an illness, such as a stomach bug. CIDP isn’t linked to illness. With GBS, once treated, most people recover fairly quickly. CIDP, on the other hand, tends to be a longer-term problem. In rare cases, people who don’t recover from GBS may develop CIDP.

How is it diagnosed?

There’s no test to diagnosis CIDP. Instead, your doctor will ask you questions about your symptoms, such as when they started and how they feel. He’ll do a thorough physical examination and may also recommend tests to get a better idea of what’s going on with your nerves, and to rule out other possible causes.

In some cases, doctors can’t be completely sure it’s CIDP, but they may go ahead and start treatment. If the symptoms improve, that’s strong evidence it’s CIDP.

Continued

What do you do to feel better?

Early treatment is key. It can help prevent nerve damage . That can help stop symptoms from becoming severe.

Treatment may include:

  • Corticosteroids. These medications bring down inflammation and slow the immune system.
  • Intravenous immunoglobulin ( IVIG ). Your doctor may give you injections of concentrated antibodies from healthy people to slow your body’s immune response.
  • Plasma exchange (PE). This treatment involves receiving a part of blood called plasma through an IV to slow down your immune system.
  • Immunotherapy. These drugs interrupt your immune system to help stop it from attacking the myelin.
  • Stem cell transplant. In rare cases, your doctor may inject healthy stem cells (either yours or donated by someone else) to “reset” your immune system.

Your doctor may also recommend physical therapy. Moderate exercise may give you more energy.

You may find your symptoms are manageable sometimes and hard to deal with at other times. If over-the-counter painkillers aren’t enough to treat your pain from CIDP, your doctor may prescribe other medications .

You may recover entirely from CIDP. Some people do, but they may have symptoms from nerve damage, such as numbness and weakness, for the rest of their lives.

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What to know about CIDP

Last reviewed

Last reviewed Fri 2 Feb 2018

Table of contents

  1. Causes
  2. Symptoms
  3. Treatments
  4. Diagnosis
  5. Diet and CIPD
  6. Takeaway
Chronic inflammatory demyelinating polyneuropathy is a neurological disease that damages and destroys the nerves in a person’s body.

The condition, which is abbreviated to CIDP, affects a person’s ability to move, especially their arms and legs, as well as their sensory functions, leading to tingling and numbness.

The disorder is rare, occurring in around 5 to 7 people per 100,000 people in the United States, according to the National Organization for Rare Disorders .

Fast facts on CIDP:

  • The average age of onset of the disease is 50 years old.
  • Men are twice as likely as women to experience it.
  • Symptoms may start with a change in walking or tingling and numbness in the hands and feet.

What causes CIDP?

brain neurons with myelin sheath.
It is believed that CIPD is caused by the body attacking the myelin sheaths that protect nerves, causing inflammation.

Although doctors do not know the exact cause of CIDP, they believe the condition is an autoimmune disorder where the body’s defense systems attack healthy tissues.

In the case of CIDP, these healthy tissues are the myelin sheaths that protect nerves and enable the nervous system to transmit signals more quickly.

The condition also causes inflammation in the nerves.

Difference to other disorders

While the disease has some similarities to other neurological disorders, such as Guillain-Barre syndrome (GBS) and multiple sclerosis (MS), there are several differences in onset, symptoms, and treatments.

For example, a person with GBS can often identify an infection that occurred before their symptoms began, such as mononucleosis. Those with CIDP cannot usually recall a preceding infection.

A person with CIDP usually has symptoms that may continue for about 8 weeks, or twice as long as the duration of typical GBS symptoms.

Another difference is that GBS is an acute disorder that will not typically recur while CIDP symptoms may be ongoing. Some doctors consider CIDP a chronic form of GBS.

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Symptoms

Symptoms associated with CIPD tend to be progressive. Some of the condition’s potential symptoms include:

  • clumsiness
  • dysphagia or difficulty swallowing
  • double vision
  • foot drop
  • loss of reflexes
  • numbness in the hands or feet
  • tingling or pain in the extremities
  • unexplained fatigue

The condition’s symptoms tend to occur on both sides of the body, for example, in both legs. Some people may only notice changes in sensory function, such as tingling and numbness, without experiencing changes in their walking or movement.

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What are the treatment options?

Intravenous medication in clear bottle next to syringe.
Intravenous medication may be used to treat symptoms of CIPD.

Treatment for CIDP involves trying to reduce the inflammation that causes the nerve-related symptoms.

Although there is no cure for the condition, medications that modulate or normalize the immune system can help improve the effects CIDP has on a person’s nerves. The U.S. Food and Drug Administration (FDA) recently approved two drugs to treat CIDP.

The selected drugs decrease the immune system activity that causes nerve-related symptoms.

Both are in the IVIg (intravenous immunoglobulin) class.

One of these drugs is Gamunex, and the other is Privigen. Both drugs contain antibodies (immunoglobulins) that help prevent other immunoglobulins from damaging nerves.

While these medications can help to reduce inflammation in CIDP, they do not cure it.

A doctor may also prescribe medications called immunomodulators that are known to suppress the immune system and improve signs and symptoms of CIDP.

These drugs include:

  • azathioprine
  • cyclophosphamide
  • cyclosporine
  • methotrexate
  • mycophenolate

Another effective option to treat CIDP is plasma exchange or plasmapheresis.

This procedure involves removing blood from an individual and separating the red blood cells and components, such as antibodies, from the plasma that contributes to CIDP. Donor plasma is then added to the blood, which is transfused back into the individual.

Some medicines can help the symptoms of CIDP without modulating the immune system or decreasing inflammation.

These drugs can be given alongside the immunomodulators mentioned above. They include:

  • carbamazepine
  • gabapentin
  • amitryptiline
  • pregabalin
  • duloxetine

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Diagnosis

physical therapist testing patients reflexes in knee joint.
A persons reflexes may be tested to diagnose CIPD, as the condition can affect reflex time and limb strength.

CIDP is a rare condition, so doctors may have difficulty in diagnosing it initially.

Because the symptoms are often progressive, a doctor may need to monitor an individual over the course of 1 to 2 months before being clear about the diagnosis.

A doctor will begin by taking a medical history and asking the person about symptoms. Some signs that may indicate CIDP include the absence of reflexes and weakness in the arms and legs.

Testing

After considering a person’s symptoms, a doctor will likely order a range of tests to rule out other similar disorders. For example, they may recommend a lumbar puncture to test spinal fluid for the presence of inflammatory cells, such as white blood cells. Meningitis and nervous system cancer can also cause a range of symptoms that are similar to CIDP.

A doctor may also order tests to measure how well a person’s nerves are conducting electrical signals. These tests include nerve conduction testing and electromyography.

If a person’s nerves are not conducting as quickly as expected, this could support a diagnosis of CIDP.

People respond differently to CIDP treatments. Some may see an improvement in immune system function, others may see many of their symptoms resolve, but some may not have any improvement.

Can diet help treat CIDP?

Sometimes doctors will recommend an anti-inflammatory diet for those with CIDP to help reduce their symptoms. However, diet is no substitute for the medications listed above.

An anti-inflammatory diet has many of the same characteristics as most healthful diets, so a person should avoid:

  • high-sodium foods
  • high-sugar foods
  • processed foods
  • saturated fats
  • trans fats

A person with CIDP should eat a predominantly plant-based diet filled with colorful fruits and vegetables. Other foods someone with CIDP should eat, include lean meats and fatty, low mercury fish, such as salmon.

Dietary recommendations may vary according to an individual’s additional health conditions or unique dietary preferences.

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Takeaway

Those treated for CIDP often experience an improvement in symptoms, then a series of relapses.

According to a study published in the journal Current Treatment Options in Neurology , an estimated 90 percent of people with CIDP will respond to immunosuppressive treatments, such as IVIg.

However, in the long-term, many people with CIDP eventually require assistive devices, such as canes, walkers, or wheelchairs to help them move around.

As a general rule, the earlier a person is diagnosed and treated for CIDP, the better their prognosis tends to be.

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    Article last reviewed by Fri 2 February 2018.

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