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.
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.
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
A diagnosis of CIDP usually requires:
- Clinical evaluation. A doctor will look at a child’s symptoms and decide what further testing for CIDP is needed.
- Nerve conduction studies (NCS). This involves measuring the nerves’ response to electrical stimulation.
Electromyography (EMG). This records signals in the muscles using a small needle.
CIDP occurs less frequently in children. Therefore, additional testing may be required in children to be sure of the CIDP diagnosis. Additional testing helps to confirm inflammation of the nerves. It can rule out other disorders that can mimic CIDP.
Common additional testing includes:
- Spinal tap. This is also known as a lumbar puncture. It will assess cerebrospinal fluid and can help confirm diagnosis. It is not always needed.
- Spinal imaging. Imaging is not always needed but may sometimes be used to clarify the diagnosis. It may be done to rule out alternative causes of CIDP. It can also be done to look for inflammation in the nerves. Two types of imaging used are:
- Magnetic resonance imaging (MRI) of the spine, spinal roots or plexus
- Nerve ultrasound
Less common testing includes:
- Nerve biopsy. A nerve biopsy tests a small sample of a nerve. It is rarely used but may be done in unusual cases.
- Antibody blood test. This can look for the antibodies that the immune system inappropriately targets that are associated with CIDP.
Many of these investigations do not need to be repeated unless symptoms significantly worsen, or the disease progresses in an unusual way. However, a doctor may repeat some of these tests to help monitor how a child is responding to treatment and how long treatment should continue.
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.
Intravenous immunoglobulin (IVIG)
IVIG is a medication given through a vein. It is usually repeated every 3 to 4 weeks. IVIG is a collection of pooled antibodies from blood donors. These antibodies appear to block the immune system from attacking the nerves. It may take 2 or 3 months to see any improvement. The most common side effects are:
In rare cases, IVIG can cause:
- Blood to abnormally clot
- Kidney issues
- Aseptic meningitis
Glucocorticoids, a type of steroid, are given as an oral medication. It can either be given as a higher dose over a few days or in a lower dose over a longer period. Occasionally, when a higher dose is needed, steroids are given through an IV. Common side effects include:
- Increased appetite
- Increased blood pressure
- Mood changes or irritability
- Thinning of the bones
Immune system suppressors
Certain varieties of CIDP or more severe cases may require other types of drugs to suppress the immune system. This may include medications such as:
- Mycophenolate mofetil
Each of these drugs has unique side effects. The most important side effect for all of them is an increased risk of infection.
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.
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
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.
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AllStripes partners with rare disease patient advocacy organizations and key opinion leaders to build the programs. Expert researchers produce original research insights and publications, as well as partner with academic institutions and pharmaceutical companies to make progress towards new treatments.
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GBS|CIDP Foundation International
The GBS|CIDP Foundation International is a global nonprofit organization supporting individuals and their families affected by Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), and related conditions through a commitment to support, education, research and advocacy. The Foundation has a Youth, Teen, and Young Adult Events page. They also host a Youth, Teen, and Young Adult private Facebook group.
Child Neurology Foundation (CNF) solicits resources from the community to be included on this webpage through an application process. CNF reserves the right to remove entities at any time if information is deemed inappropriate or inconsistent with the mission, vision, and values of CNF.
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 GBS|CIDP Foundation International believes that sharing stories can help the healing. The Patient Stories page is a good place to start that process by reading about others, including children, who are living with Chronic Inflammatory Demyelinating Polyneuropathy and other similar conditions. You may also submit your story via that same Patient Stories page.
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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