Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disease that damages and destroys the nerves in a person’s body.
CIDP affects a person’s ability to move, especially their arms and legs, as well as their sensory functions, leading to tingling, numbness, and diminished sensation.
The disorder is rare, occurring in around 5–7 people per 100,000 in the United States.
This article discusses CIDP in more detail, including causes, diagnosis, symptoms, and treatment.
CIDP fast facts
- The average age of onset of CIDP is 50 years old.
- Males are twice as likely as females to have the condition.
- Symptoms may start with a change in walking or tingling and numbness in the hands and feet.
Although experts do not know the exact cause of CIDP, they believe it 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 the peripheral nerves and enable the nervous system to transmit signals more quickly. The condition also causes inflammation in the nerves.
While the disease has some similarities to other neurological disorders, such as Guillain-Barré 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
Another difference is that GBS is an acute disorder that will not typically recur, while CIDP symptoms may be ongoing or recurrent. Some doctors consider CIDP a chronic form of GBS.
When compared with MS, both are demyelinating conditions that can cause recurrent or ongoing symptoms. However, MS causes demyelination of the central nervous system — the brain, spinal cord, and optic nerves — while CIDP causes demyelination of the peripheral nervous system.
CIDP is rare, so doctors usually initially consider more common neurological conditions before diagnosing CIDP.
As the symptoms are often progressive, a doctor may need to monitor an individual 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, diminished sensation, and weakness in the arms and legs.
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. Many people experience partial or complete resolution of symptoms, but some may not have any improvement.
Symptoms associated with CIDP tend to be
- clumsiness
- dysphagia or difficulty swallowing
- double vision
- foot drop
- numbness in the hands or feet
- tingling or pain in the extremities
- loss of sensation in the arms or legs
- 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. These symptoms usually develop gradually and progress over days or sometimes weeks.
Treatment for CIDP involves aiming to reduce the inflammation and demyelination that causes the nerve-related symptoms.
Although there is no cure for the condition, medications that modulate or standardize the immune system can help improve the effects CIDP has on a person’s nerves. The Food and Drug Administration (FDA) has approved two drugs to treat CIDP: Gamunex and Privigen.
These drugs decrease the immune system activity that causes nerve-related symptoms. Both are in the IVIg class, meaning that immunoglobulins are injected intravenously over several hours. It works by reducing the effect of the antibodies causing the condition.
Both Gamunex and Privigen contain antibodies — immunoglobulins — that help prevent other immunoglobulins from damaging myelin and nerves.
Newer treatments with preparations of higher concentrations of immunoglobulins are available. This can be given under the skin — subcutaneously.
While these medications can help to reduce inflammation in CIDP, they do not cure it.
Another new treatment option is
A doctor
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 and transfused back into the individual.
Some medications can help uncomfortable sensory symptoms of CIDP without modulating the immune system or decreasing inflammation.
A person can take these drugs alongside the immunomodulators mentioned above. They include:
- carbamazepine
- gabapentin
- amitryptiline
- pregabalin
- duloxetine
Sometimes, doctors will recommend an anti-inflammatory diet for those with CIDP to help reduce the recurrence of inflammation. However, diet alone is not sufficient to substitute for the medications listed above.
An anti-inflammatory diet has many of the same characteristics as most healthy diets, so a person should avoid:
- high sodium foods
- high sugar foods
- processed foods
- saturated fats
- trans fats
A person with CIDP may benefit from a predominantly plant-based diet filled with colorful fruits and vegetables. Other beneficial foods 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.
Those treated for CIDP often experience an improvement in symptoms, then a series of relapses.
A 2018 study found that the overall response rate among people with CIDP who received conventional first-line immunotherapies was
However,
As a general rule, the earlier a person receives a diagnosis and treatment for CIDP, the better their outlook tends to be.
CIDP is a rare disease that affects a person’s mobility and sensory functions, causing symptoms such as tingling, numbness, and loss of sensation in the limbs. The cause is still unclear, but experts believe it is related to the immune system’s attack on the myelin sheaths protecting the peripheral nervous system.
CIDP shares some similarities to other neurological disorders, such as GBS and MS. It is important to get the correct diagnosis to receive proper treatments. Many types of treatments are available, some of which can lead to long-term remission. However, CIDP does not have a cure to date, and a person with this condition may expect to experience relapses and live with some degree of physical disability.