What is Chronic Inflammatory Demyelinating Polyneuropathy & How is it Treated?

What is Chronic Inflammatory Demyelinating Polyneuropathy?

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare type of neurological condition that causes swelling or inflammation of the nerves(1,2,3,4) This inflammation damages or destroys the protective wrapping that covers the nerves and fibers, known as myelin or the myelin sheath. Once this fatty coating gets destroyed, chronic inflammatory demyelinating polyneuropathy can cause symptoms like tingling sensations or numbness in the hands and feet, along with loss of muscle strength. (5,6)

Chronic inflammatory demyelinating polyneuropathy is often known to be a type of acquired immune-mediated inflammatory disorder, which means that it is not a contagious condition, but it is a chronic disease. The disease is long-term, and once you have it, you are likely to live with the symptoms and complications of this condition for the rest of your life.

Chronic inflammatory demyelinating polyneuropathy, just like the other similar inflammatory disorders, has an impact on your peripheral nervous system. The peripheral nervous system includes the nerves outside your spinal cord and brain. Knowing the different symptoms of the disorder and how they can progress with time can help you, and your doctor determine the best treatment option for your condition. (7,8)

According to the National Organization for Rare Disorders, chronic inflammatory demyelinating polyneuropathy is so rare that it only occurs in just 5 to 7 people per 100,000 people in the United States. (9) Here are some facts on the disease:

  • The average age of onset of chronic inflammatory demyelinating polyneuropathy is 50 years old.
  • Men are two times more likely to get this disease as compared to women.
  • Symptoms typically begin with a change in walking or experiencing slight numbness and tingling in the hands and feet.

What are the Symptoms of Chronic Inflammatory Demyelinating Polyneuropathy?

The symptoms of chronic inflammatory demyelinating polyneuropathy tend to be progressive. The common symptoms may include:

  • Slowed Nerve Response: As the myelin sheath gets destroyed and lost over time, the electrical impulses between the brain and your nerves start to get slowed down or may get lost altogether. In the starting, the damage may be so small that you are unlikely to even notice any difference. Over time, though, the slow nerve response will start becoming quite noticeable.
  • Sensory Changes: Burning, tingling, and numbness can develop, and you may start to notice changes to your senses, like reduced sensitivity to touch, loss of taste, and more.
  • Loss of Reflexes: Muscles may stop responding as quickly as they used to, and you may also notice some amount of muscle weakness.
  • Symmetrical Symptoms: Most people with chronic inflammatory demyelinating polyneuropathy tend to experience identical symptoms on both sides of the body. So instead of experiencing tingling in just one hand or foot, you are likely to experience it in both the hands and feet at the same time.
  • Slow Development Of The Symptoms Over A Long Time: Symptoms of chronic inflammatory demyelinating polyneuropathy will increase in severity slowly over the course of few months or even years. This slow progression of the disease might not even be detectable at first, and many people will continue to live with the symptoms for a long time before getting a proper diagnosis made.

Some other symptoms of chronic inflammatory demyelinating polyneuropathy may include:

  • Double vision
  • Clumsiness
  • Difficulty swallowing or dysphagia
  • Foot drop
  • Tingling or pain in the hands and feet
  • Unexplained fatigue
  • Numbness in the hands and feet

What are the Causes of Chronic Inflammatory Demyelinating Polyneuropathy?

Scientists and doctors are not clear about the exact causes of chronic inflammatory demyelinating polyneuropathy, but it is believed to develop as a result of an abnormal immune response. This is believed to be an autoimmune disorder, where the body’s immune system starts to attack the healthy tissues. In the case of chronic inflammatory demyelinating polyneuropathy, the healthy tissues are the myelin sheaths that cover and protect the nerves and allow the nervous system to transmit signals more quickly. Chronic inflammatory demyelinating polyneuropathy damages or destroys the nerves, which in turn causes inflammation in the nerves. (10,11)

How is Chronic Inflammatory Demyelinating Polyneuropathy Different From Other Disorders?

Chronic inflammatory demyelinating polyneuropathy has several similarities to other neurological conditions, especially multiple sclerosis, and Guillain-Barre syndrome. At the same time, there are many differences in the symptoms, onset, and treatments.

For instance, a person with Guillain-Barre syndrome can usually identify an infection that developed before their symptoms began, such as mononucleosis. Those with chronic inflammatory demyelinating polyneuropathy, though, cannot generally recall a preceding infection.

A person with chronic inflammatory demyelinating polyneuropathy also has symptoms that tend to continue for approximately eight weeks, or twice as long as the duration of the common symptoms of Guillain-Barre syndrome.

Many researchers believe that chronic inflammatory demyelinating polyneuropathy is the chronic form of Guillain-Barre syndrome. Both conditions also develop the symptoms in a symmetrical pattern, and the symptoms of both these conditions tend to begin in the fingers or toes and then move on to affect the bigger muscles. (12,13)

It is important to know that neither Guillain-Barre syndrome nor chronic inflammatory demyelinating polyneuropathy cause damage to your central nervous system, and neither do they affect your mental cognition. However, unlike chronic inflammatory demyelinating polyneuropathy, the symptoms of Guillain-Barre syndrome show up quickly and reach emergency levels very soon. Most people with Guillain-Barre syndrome require intensive care, while the symptoms of chronic inflammatory demyelinating polyneuropathy develop slowly. Hospital care in chronic inflammatory demyelinating polyneuropathy is only necessary once the disease progresses quite a lot and starts to severely restrict your daily activities. (14)

The onset of Guillain-Barre syndrome happens quickly and also disappears equally quickly. The lasting effects of any damage caused by Guillain-Barre syndrome can take several months or even years to get adjusted to, but usually, the symptoms don’t get worse. Also, the Guillain-Barre syndrome is unlikely to recur. Only five percent of people with Guillain-Barre syndrome experience the symptoms again.

When it comes to multiple sclerosis, just like chronic inflammatory demyelinating polyneuropathy, the myelin sheath around the nerves gets destroyed in this disorder. Multiple sclerosis is also a progressive and chronic disease, and it has a gradual progression, meaning the symptoms worsen over time. Some people may experience some periods of stability followed by periods of flare-ups or relapse. (15)

Unlike chronic inflammatory demyelinating polyneuropathy, people with multiple sclerosis develop plaques on their spinal cord and brain. (16) These plaques prevent the nerves from functioning properly, and they are not able to transmit the signals from the brain to the central nervous system and to the rest of the body. Over a period of time, multiple sclerosis may even start attacking the nerves themselves, thus destroying them. Symptoms of multiple sclerosis tend to show up on one side of the body at a time and not in a symmetrical pattern. The impacted area and the severity of symptoms depend on which nerves the disease attacks. (17,18)

How is Chronic Inflammatory Demyelinating Polyneuropathy Diagnosed?

Chronic inflammatory demyelinating polyneuropathy is a complex condition to diagnose. It is a very rare disorder, and doctors try to rule out the more common conditions or disorders first before reaching a diagnosis of chronic inflammatory demyelinating polyneuropathy.

To diagnose chronic inflammatory demyelinating polyneuropathy, doctors will run various tests and physical exams. Firstly, your doctor will take down a detailed medical history and will ask you questions about your symptoms, when they started, and how or if the symptoms have changed. For diagnosing chronic inflammatory demyelinating polyneuropathy, you need to have experienced the symptoms for at least eight to ten weeks.

Additionally, your doctor will also order numerous diagnostic tests to examine other parts of the body. It is common for a doctor to prescribe a nerve conduction test to check how quickly the nerve impulses travel throughout the body. (19) The results of this test will help your doctor in the future to find out if your impulses have gotten worse or if they are improving.

A spinal fluid analysis may also be done, along with urine and blood tests, to rule out other possible causes and underlying conditions that could be causing your symptoms.

How is Chronic Inflammatory Demyelinating Polyneuropathy Treated?

Once you have been diagnosed with chronic inflammatory demyelinating polyneuropathy, your doctor will first recommend that you visit a specialist. Doctors who specialize in the treatment of immune-mediated disorders or autoimmune diseases have more experience in treating such conditions. Lifestyle changes will also help slow down the progression of the disorder and allow you to adapt to changes more easily.

Treatment for chronic inflammatory demyelinating polyneuropathy revolves around reducing the inflammation that causes your symptoms.

It is essential to know that there is no cure for chronic inflammatory demyelinating polyneuropathy. However, medications that normalize or modulate the immune system can help improve some of the effects of chronic inflammatory demyelinating polyneuropathy on your nerves. The US Food and Drug Administration has recently approved two drugs for treating chronic inflammatory demyelinating polyneuropathy. (20)

The two drugs reduce the activity of your immune system that causes the nerve-related symptoms in chronic inflammatory demyelinating polyneuropathy. Both the drugs belong to the intravenous immunoglobulin (IVIg) class. One of the drugs is Privigen, and the other is Gamunex. Both medications contain immunoglobulins (antibodies) that help stop other immunoglobulins from damaging your nerves. (21,22)

While these drugs can help lower the inflammation in chronic inflammatory demyelinating polyneuropathy, but they will not cure the disease.

Your doctor may also prescribe other medications known as immunomodulators that help suppress the immune system and also improve the symptoms of the disorder. These medications include:

  • Mycophenolate
  • Cyclosporine
  • Methotrexate
  • Azathioprine
  • Cyclophosphamide

Another effective treatment option is plasmapheresis or plasma exchange. This process involves removing the blood from a person and then separating the red blood cells and components like antibodies from the plasma that contributes to chronic inflammatory demyelinating polyneuropathy. In some cases, donor plasma is also added to the blood and transfused back into the person.

Certain medications can help improve the symptoms of chronic inflammatory demyelinating polyneuropathy without needing to modulate the immune system or reducing inflammation. These medications can also be prescribed along with immunomodulators. These include:

  • Pregabalin (23)
  • Duloxetine
  • Carbamazepine
  • Amitryptiline
  • Gabapentin (24)

Can Exercise and Diet Help in Chronic Inflammatory Demyelinating Polyneuropathy?

Research shows that people with chronic inflammatory demyelinating polyneuropathy can benefit from resistance exercise training and aerobic exercise training. (25) Both these types of exercises help improve muscle strength and lung capacity. Building your muscle endurance and strength can help decrease the effects of the disease’s progressive muscle destruction.

However, it is important to talk to your doctor before you start exercising. This is because your need to adopt an exercise regimen that works best for your fitness level. Overexerting yourself may cause damage to your muscles, which can slow down the recovery process and aggravate the symptoms of chronic inflammatory demyelinating polyneuropathy.

Along with exercise, your doctor will also recommend that you follow an anti-inflammatory diet to help reduce the severity of your symptoms. However, at the same time, you must realize that diet and exercise are not substitutes for medications.

An anti-inflammatory diet has many of the same features as most healthy diets, but at the same time, it helps reduce inflammation in the body. Your doctor will also recommend that you avoid the following:

  • High-sodium foods
  • Processed foods
  • High-sugar foods
  • Saturated and trans fats

A person with chronic inflammatory demyelinating polyneuropathy should try to consume more of a plant-based diet that includes fruits and vegetables of different colors. You should also increase the intake of fatty, low mercury fish like salmon and lean meats.

Dietary guidelines also vary according to any additional health conditions or dietary preferences.

Conclusion

Every person living with chronic inflammatory demyelinating polyneuropathy has a different outlook. It is challenging to have a good quality of life with this disease, and your day-to-day life will vary depending on your symptoms. Some people tend to experience a spontaneous recovery and show some signs of the disease from time to time. In contrast, others experience partial recovery periods while having a slow and steady progression of the disease.

The best way of improving your outlook is to show a doctor at the earliest so that you can get an early diagnosis and start treatment right away. Remember that chronic inflammatory demyelinating polyneuropathy is a complex condition to diagnose, and this makes it equally tricky to quickly find a treatment that works for you. The sooner you start treatment, the more likely are the chances of preventing further damage to your nerves.

References:

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