Chronic Inflammatory Demyelinating Polyradiculoneuropathy
What is chronic inflammatory demyelinating polyradiculoneuropathy?
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare type of autoimmune disorder. In an autoimmune disease, the body attacks its own tissues. In CIDP, the body attacks the myelin sheaths — the fatty covering on the fibers that insulate and protect your body’s nerves.
Experts think that CIDP is related to the more commonly known disease Guillain-Barre syndrome (GBS). But while GBS is generally considered more of an acute, or short-term, disease, CIDP is considered a chronic, or long-term, disease. CIDP is less common than Guillain-Barre syndrome.
CIDP is usually classified as follows:
Progressive. The disease continues to worsen over time
Recurrent. Episodes of symptoms that stop and start
Monophasic. One bout of the disease that lasts 1 to 3 years and doesn’t recur
What causes chronic inflammatory demyelinating polyradiculoneuropathy?
CIDP occurs when the body's immune system attacks the myelin sheaths around nerve cells, but exactly what triggers this is not clear. Unlike Guillain-Barre syndrome, there is usually no infection preceding CIDP. There does not seem to be a genetic link to CIDP.
What are the risk factors for chronic inflammatory demyelinating polyradiculoneuropathy?
Though CIDP can occur in anyone, people in their 50s and 60s seem more likely to develop it than other age groups. Men are twice as likely as women to get the disease.
What are the symptoms of chronic inflammatory demyelinating polyradiculoneuropathy?
Regardless of the type of CIDP you may have, symptoms are usually the same, and can include:
- Tingling in your arms and legs
- Gradual weakening of your arms and legs
- Loss of reflexes
- Loss of balance and your ability to walk
- Loss of feeling in your arms and legs, which often starts with your inability to feel a pin prick
How is chronic inflammatory demyelinating polyradiculoneurypathy diagnosed?
Because CIDP is a rare condition, it’s often difficult for doctors to correctly diagnose the disease, at least at first. Doctors can confuse its symptoms with those of GBS, because of the similarity between the two diseases. If symptoms last longer than 8weeks, your doctor may suspect CIDP.
After taking your medical history and doing a physical exam, your health care provider may perform additional tests to confirm a diagnosis including:
- Blood and urine tests
- A nerve conduction study to look for myelin damage in peripheral nerves. This test is sometimes called an electromyogram. It involves using low electrical currents to test nerve function and response.
- A lumbar puncture. In this procedure, a small needle is inserted into your back and a sample of your cerebrospinal fluid (CSF) is withdrawn. CSF is the fluid that surrounds your spinal cord. The procedure helps doctors detect elevated levels of certain proteins related to the disease.
How is chronic inflammatory demyelinating polyradiculoneuropathy treated?
Treatment for CIDP is often effective. Some studies show that up to 80% of people respond well to therapy. Because it is an autoimmune disorder, doctors use medications that suppress your immune response to treat CIDP. Your medical team tailors your treatment to you and closely monitors your progress. Treatments for CIDP include immunosuppressive drugs, steroids, intravenous immunoglobulin, and plasma exchange (plasmapheresis) to remove immune system proteins from the blood.
Living with chronic inflammatory demyelinating polyradiculoneuropathy
The course of CIDP can vary greatly between different people, as can the response to treatment.
Getting treatment as early as possible is very important because it gives you the best chance of limiting symptoms and keeping this condition under control. If you do not seek treatment for CIDP, your symptoms will likely progress over the course of several years. These can range from sensory symptoms, such as tingling and numbness, to weakness and loss of balance. Without treatment, 1 in 3 people with CIDP will need a wheelchair.
In people with permanent physical impairments, physical therapy can be very important. In this treatment, specialists will work with you to maintain or increase your strength and improve your coordination. Another type of therapy is occupational therapy, which helps you learn new ways of doing everyday tasks in spite of your new physical limitations.
Some people with physical disabilities often feel sad or depressed. If this happens to you, your doctor may recommend that you see a mental health professional. Antidepressant medications and psychotherapy can help treat depression. So can support groups for people managing chronic health conditions.
When should I call my health care provider?
If you have been diagnosed with CIDP, talk with your health care provider about when you might need to call them. They will likely advise you to call if you notice worsening of any symptoms or if you develop any new symptoms.
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a slowly developing autoimmune disorder in which the body's immune system attacks the myelin that insulates and protects your body’s nerves. The exact cause is not known.
- Common symptoms are gradual weakness or sensation changes in the arms or legs. This might get worse over time, or it might come and go.
- Early treatment is important to limit disease progression, and includes medicines or other treatments to suppress the immune system.
Tips to help you get the most from a visit to your health care provider:
- Before your visit, write down questions you want answered.
- Bring someone with you to help you ask questions and remember what your provider tells you.
- At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you.
- If you have a follow-up appointment, write down the date, time, and purpose for that visit.
- Know how you can contact your provider if you have questions.
- Bass III, Pat F., MD, MPH
- Fraser, Marianne, MSN, RN