Authors: Amanda Yaworski, MD; Jenna Klotz, MD, Stanford University

Reviewed: November 2021

SUMMARY

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a disorder that affects the peripheral nerves. CIDP is an acquired disease. This means that children are not born with CIDP. Rather, they develop the disease at some point later on.

CIDP is considered an autoimmune disease. The body’s immune system prevents and treats infections in the body. However, in autoimmune disorders, the immune system mistakes a normal, healthy part of the body for an infection. Due to an abnormal immune response, there is the production of autoantibodies, antibodies that mistakenly attack a part of the body, in addition to attacking the infection. In CIDP, certain parts of the nervous system, specifically the nerves, are attacked and injured.

In a usual case of CIDP, children will have weakness on both sides of the body. This weakness can involve both the:

• Muscles that are closer to the torso, like shoulder and hip muscles
• Muscles that are further away from the torso, like finger muscles

CIDP may also cause sensory changes, such as a feeling of numbness or tingling.

Disorder Overview

DESCRIPTION 

CIDP causes damage to the nerve roots and nerves that control movement and, sometimes, sensation. Nerves are like wires that send signals to your muscles so they can move. They have an inner part, called the axon, and a coating, called the myelin sheath. The myelin sheath insulates the nerves and helps the signal travel more efficiently. CIDP typically damages the myelin sheath. This is known as demyelination. In some cases, CIDP can mostly damage the axon instead. The axon connects neurons together.

A typical case of CIDP causes weakness on both sides of the body that gets progressively worse over at least 8 weeks. This is known as symmetric weakness. CIDP can also cause changes in sensation or feeling, resulting in tingling or numbness. There are some less common variants of CIDP. These other variants may have slightly different sets of symptoms:

• Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM)
• Sensory-predominant CIDP
• Distal acquired demyelinating symmetric (DADS) neuropathy
• Pure motor CIDP
• Neurofascin antibody-mediated CIDP

CIDP symptoms can vary significantly from very mild to severe. Milder cases of CIDP may cause mild weakness. In these cases, children may trip more often or have trouble with buttons or zippers. More severe cases may make it difficult to do daily tasks of living, like walking or eating.

CIDP does not usually cause death or shorten the life span.

SIGNS AND SYMPTOMS 

Symptoms of CIDP are usually progressive. This means that they will continue to worsen for at least 8 weeks.

The usual symptoms of CIDP include:

• Muscle weakness. Muscle weakness in CIDP worsens slowly and occurs on both sides of the body. It usually includes both:
  • Proximal muscles. These are muscles closer to the torso, such as hip or shoulder muscles.
  • Distal muscles. These are muscles further from the torso, such as finger or ankle muscles.
• Sensory changes. These might include:
  • Sensations of tingling. May feel like pins and needles.
  • A loss of feeling is also reported.
• Movement issues. An unsteady gait or frequent tripping can cause falls.

CIDP can also sometimes cause a mild tremor in children.

CIDP usually begins very gradually over 8 weeks or more. However, in some cases, symptoms come on more quickly. Weakness and sensory changes may become more severe as the disease progresses, but they should improve once treatment is started.

CAUSES

CIDP is not caused by anything that a parent did or did not do. There is no known genetic cause, either. In fact, the exact cause of CIDP is not known. However, there is evidence that it is autoimmune. It is likely related to an abnormality within the immune system. In CIDP, an unknown trigger activates the immune system, which mistakenly attacks the nerves.

Sometimes, a blood test can show certain antibodies that the immune system is producing that is causing the nerve damage. However, in most patients, these antibodies are never found.

DIAGNOSIS AND LABORATORY INVESTIGATIONS

TREATMENT AND THERAPIES

The goal of treatment in CIDP is to prevent the immune system from attacking the nerves. Treatment will vary depending on:

• A child’s symptoms
• How much symptoms are impacting everyday functioning
• The stage of the disease

Treatment usually consists of medication. Unfortunately, it is difficult to predict how each patient will respond to a specific medication. For this reason, patients may need to try multiple medications before the right one is found.

Most patients are initially treated with either IVIG or steroids. However, other options are sometimes used, too.

Most patients with CIDP require treatment for a few months before there is any improvement. Medication is tapered off slowly. The goal is that the patient will not need long-term medication. However, for some patients, this is not possible. A lower dose of long-term medication may be needed.

If CIDP is not treated, symptoms generally continue to worsen. With treatment, the symptoms in some children will resolve completely. There are some side effects to CIDP treatment. However, most patients tolerate them well overall. The improvement in symptoms usually outweighs any side effects.

OUTLOOK

When treated, most children with CIDP will significantly improve. About 30% will be cured or go into remission. Remission means having no symptoms for at least 5 years.

Upon first being treated, most children will have improvement, but 10% to 15% will not respond. About half of children will not have any new symptoms after treatment is finished. This is known as a monophasic course. The other half will still have symptoms that come and go and may require ongoing treatment. This is known as a relapsing and remitting course.

Impact on Daily Life

Over 80% of children with CIDP will be fully independent as adults. If CIDP symptoms are mild, they may not impair daily activities at all.

However, if more severe, some things may become more difficult. Since CIDP causes weakness, it may be harder to:

• Participate in certain sports or activities, like riding a bike or participating in physical education
• Use buttons or zippers
• Hold a fork
• Walk

Sensory changes may also make balance more difficult.

CIDP doesn’t seem to affect the way children grow or learn, or their general intelligence.

In addition to affecting physical activity, children may have some disruption to their usual schedules. They may need to: 

• Receive IV medications at an infusion center or hospital or at home
• Work with physical or occupational therapists to help regain strength
• Attend more doctor’s visits

Impact on Education

Depending on degree of disability:

• Physical education may need to be modified
• Children may need more time getting from class to class
• Stairs may be difficult

Diet and Exercise in CIDP

It is important for children with CIDP to continue with physical activity, exercise, and stretching. This can help to strengthen some of the weak muscles. Stretching also prevents the formation of contractures. Contractures can form when a joint gets stuck in an abnormal position due to weakness.

A healthy diet is always helpful. However, there are no specific changes to the diet that need to be made for children with CIDP. Children on steroids may have increased appetite and weight gain. Working with a pediatrician or dietitian to ensure healthy eating habits can be especially helpful in these cases.

RELATED DISORDERS

Disorders with Similar Symptoms

• Guillain-Barre syndrome (GBS). GBS works in a way similar to CIDP but usually happens faster and does not require long-term treatment with immune therapy. GBS does not usually cause persistent damage to the nerves like CIDP. About 5% of patients with GBS have more than one episode. If they have three or more episodes, their doctors may do additional testing to see if they actually have CIDP instead. CIDP is most similar to one common subtype of GBS called acute inflammatory demyelinating polyneuropathy (AIDP).
• Genetic disorders. If there is a strong family history of nerve problems in someone with CIDP symptoms, their doctor may also look for genetic causes. One example of a genetic disorder that can mimic CIDP is Charcot-Marie-Tooth (CMT) disease.
• Metabolic disorders. If a child suspected of having CIDP does not respond to the typical treatments used in CIDP, their doctor may look for metabolic disorders. These disorders can mimic CIDP early on. In metabolic disorders, the body processes proteins, fats, or sugars in an abnormal way. This can lead to a buildup of toxins in the body that can damage nerves.

CIDP Variants 

There are several variants of CIDP, as listed above. They are less common than the classic demyelinating form. The most common types of CIDP in children are:

• The classic variant, which affects the myelin
• The axonal variant, which affects the axons, sometimes called CIAP (chronic inflammatory axonal polyradiculoneuropathy)

Research 

ClinicalTrials.gov for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (birth to 17 years)

These are clinical trials that are recruiting or will be recruiting. Updates are made daily, so you are encouraged to check back frequently.

ClinicalTrials.gov is a database of privately and publicly funded clinical studies conducted around the world. This is a resource provided by the U.S. National Library of Medicine (NLM), which is an institute within the National Institutes of Health (NIH). Listing a study does not mean it has been evaluated by the U.S. Federal Government. Please read the NLM disclaimer for details.   

Before participating in a study, you are encouraged to talk to your health care provider and learn about the risks and potential benefits.  

The information in the CNF Child Neurology Disorder Directory is not intended to provide diagnosis, treatment, or medical advice and should not be considered a substitute for advice from a healthcare professional. Content provided is for informational purposes only.  CNF is not responsible for actions taken based on the information included on this webpage. Please consult with a physician or other healthcare professional regarding any medical or health related diagnosis or treatment options. 

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