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.
JUMP TO
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
Diagnostic Testing
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.
Additional Testing
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:
- Headache
- Fever
- Nausea
In rare cases, IVIG can cause:
- Blood to abnormally clot
- Kidney issues
- Aseptic meningitis
Steroids
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
Plasma exchange
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:
- Rituximab
- Mycophenolate mofetil
- Methotrexate
- Cyclophosphamide
- Azathioprine
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.
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)
Resources
Organizations
AllStripes
The mission of AllStripes is to unlock new treatments for people affected by rare disease. Serving as a research platform dedicated to rare diseases, AllStripes makes it easy for patients to contribute to new treatment research from home. There is no cost for patients, families, or advocacy organizations to participate, join research programs, or use the platform. AllStripes does the work to collect and analyze de-identified medical records to help power faster, better drug development. By bringing patients, doctors, and researchers together everyone learns from each other’s experiences to effectively push for better treatments across all rare conditions. They have over 40 active rare disease programs, which includes CIDP, and they are adding more every month.
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.
Disclaimer: AllStripes is a Public Benefit Corporation funded by private investors. Child Neurology Foundation does not specifically endorse products provided by AllStripes.
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.
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.
Family Stories
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.
References
Silwal A, Pitt M, Phadke R, Mankad K, Davison JE, Rossor A, DeVile C, Reilly MM, Manzur AY, Muntoni F, Munot P. Clinical spectrum, treatment and outcome of children with suspected diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy. Neuromuscul Disord. 2018 Sep;28(9):757-765. https://doi.org/10.1016/j.nmd.2018.06.001. Epub 2018 Jun 12. PMID: 30072201; PMCID: PMC6509554.
Bunschoten C, Jacobs BC, Van den Bergh PYK, Cornblath DR, van Doorn PA. Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Lancet Neurol. 2019 Aug;18(8):784-794. https://doi.org/10.1016/S1474-4422(19)30144-9. Epub 2019 May 7. PMID: 31076244.
Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist-Markens PH, Cornblath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Sommer C, Topaloglu HA. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force—second revision. J Peripher Nerv Syst. 2021 Sep;26(3):242-268. https://doi.org/10.1111/jns.12455. Epub 2021 Jul 30. PMID: 34085743.
Kostera-Pruszczyk A, Potulska-Chromik A, Łukawska M, Lipowska M, Hoffman-Zacharska D, Olchowik B, Figlerowicz M, Kanabus K, Rosiak E. Pediatric CIDP: Diagnosis and management. A single-center experience. Front Neurol. 2021 Jul 2;12:667378. https://doi.org/10.3389/fneur.2021.667378. PMID: 34276534; PMCID: PMC8284159.
Koike H, Katsuno M. Pathophysiology of chronic inflammatory demyelinating polyneuropathy: Insights into classification and therapeutic strategy. Neurol Ther. 2020 Dec;9(2):213-227. https://doi.org/10.1007/s40120-020-00190-8. Epub 2020 May 14. PMID: 32410146; PMCID: PMC7606443.
Cao Y, Menon P, Ching-Fen Chang F, Mahant N, Geevasinga N, Fung VS, Vucic S. Postural tremor and chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2017 Mar;55(3):338-343. https://doi.org/10.1002/mus.25253. Epub 2016 Dec 2. PMID: 27421831.
Stino AM, Naddaf E, Dyck PJ, Dyck PJB. Chronic inflammatory demyelinating polyradiculoneuropathy—diagnostic pitfalls and treatment approach. Muscle Nerve. 2021 Feb;63(2):157-169. https://doi.org/10.1002/mus.27046. Epub 2020 Sep 11. PMID: 32914902.
Rogers AB, Zaidman CM, Connolly AM. Pulse oral corticosteroids in pediatric chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2020 Dec;62(6):705-709. https://doi.org/10.1002/mus.27058. Epub 2020 Oct 2. PMID: 32893348.