Chronic Inflammatory Demyelinating Polyneuropathy - Disease Progression, Diagnosis, and Medical Management

What Is Chronic Inflammatory Demyelinating Polyneuropathy?

It is a rare type of nerve disorder (neuropathy), with a gradually progressing weakness and loss of sensation in the individual. Chronic inflammatory demyelinating polyneuropathy is an autoimmune disease, which means one’s body mistakenly attacks one’s own nerve cells next to the skin (peripheral nerves), specifically damaging the myelin coating around them. This stops the nerve from receiving and delivering messages properly. Chronic inflammatory demyelinating polyneuropathy symptoms start slowly and progress over months and years, worsening as time goes on. It can affect an individual's quality of life, mobility, and social activity. Early treatment can promote recovery, but the condition is prone to relapses.

What Are the Causes of Chronic Inflammatory Demyelinating Polyneuropathy?

Nerves in the body help one feel and move. A healthy nerve is covered and protected by a substance called myelin which helps it send and receive signals easily across the body. Myelin works as insulation around the nerve fiber, just like the coating on an electric wire. In chronic inflammatory demyelinating polyneuropathy, the body’s own immune system recognizes the nerve as a foreign substance. It starts attacking the cells with the body’s defense mechanisms and causes damage to the myelin sheath. When the myelin covering gets damaged, the nerve cannot send its signal properly. As a result, all the electrical signals sent through the nerve fibers get delayed in reaching their destination in the body or get lost on their journey.

The underlying cause of chronic inflammatory demyelinating polyneuropathy is unknown. However, doctors confirm it is not passed on from one generation to the next (not hereditary) or spread from another affected individual (not contagious). One may suffer relapses of chronic inflammatory demyelinating polyneuropathy, requiring treatment every time. Rarely, an untreated individual affected by Guillain-Barre syndrome (a similar condition resulting from previous infections) may develop chronic inflammatory demyelinating neuropathy later.

What Are the Signs and Symptoms Associated With Chronic Inflammatory Demyelinating Polyneuropathy?

The symptoms usually progress steadily for the first eight weeks. Out of the blue, the individual may grow weak in a way they cannot do the things like walking, running, or jogging they used to do before. In the early stages of a chronic inflammatory demyelinating neuropathy, one may feel:

• Weak and tired, has difficulty walking or climbing the stairs. One may also suffer a ‘foot drop’ (dragging the feet while walking).

• Get weak in the muscles of the shoulders and arms.

• Unusually slow response and reactions (slow reflexes).

• Feeling numbness and tingling in the body.

• And one may get confined to a wheelchair for the rest of one’s life.

How to Diagnose Chronic Inflammatory Demyelinating Polyneuropathy?

Diagnosing chronic inflammatory demyelinating polyneuropathy can be difficult as its symptoms are similar to many other neurological conditions. In many cases, diagnosis and treatment may get delayed due to this. The doctor will want to know all about the symptoms, like, how did they start, how did they progress, and for how long. And conduct a few tests, such as:

1. A comprehensive neurological examination involving activities to evaluate the functional ability of muscle groups, perception of different sensations, ability to walk, coordinate and balance, and body’s response to different stimulations (reflexes). This gives an idea about the individual’s level of functional impairment and what is the current status of chronic inflammatory demyelinating polyneuropathy.

2. Lumbar puncture or spinal taping procedure, where the doctor inserts a needle into the lower back area of one’s spine to access the spinal column. And draws a sample of the clear fluid existing around the brain and spinal cord (cerebrospinal fluid). This fluid is analyzed in the lab to look for an increased amount of proteins, which may be indicative of chronic inflammatory demyelinating polyneuropathy.

3. Nerve conduction velocity study to evaluate the speed and strength of electrical signals being passed through the nerves. Delayed and diminished signals may be indicative of progressing chronic inflammatory demyelinating polyneuropathy.

4. An electromyogram (EMG) test to evaluate the signal transmission from the nerve to different muscle groups in the body to check their strength and functional ability.

5. Blood tests and urine tests look for certain proteins that may be indicative of ongoing autoimmune reactions in the body. And also to rule out other neurological conditions with similar symptoms.

What Is the Treatment for Chronic Inflammatory Demyelinating Polyneuropathy?

Early diagnosis is key for chronic inflammatory demyelinating polyneuropathy for starting the treatment in its beginning stages. If left untreated, the nerve cells may keep losing their part called axons which form the nerve fibers. Once the axons are damaged, the nerve loses its functional ability. This process cannot be reversed, and so it must be prevented by early diagnosis and treatment. Some forms of chronic inflammatory demyelinating polyneuropathy are treatable, and some are not. Identifying the affected type of condition is crucial so that one can make every effort to get it treated. The following are the current treatment modalities in use:

• Intravenous immunoglobulins (IV Ig) are the most effective drug currently available to treat chronic inflammatory demyelinating polyneuropathy. In the initial stage of the treatment, It is given to the affected individual through the veins. And it is proven to reduce disability and improve functions both in children and adults. Later on, the doctor may change it to the subcutaneous type which can be administered under the skin. Recovery from this therapy depends on the severity of the condition. It may take days to weeks or longer than that.

• Plasma exchange or plasmapheresis (PLEX) treatment, where the harmful proteins that damage the nerves are removed from the body by exchanging the clear fluid portion of the blood known as plasma.

• Corticosteroids such as Prednisone or Prednisolone can reduce the body's inflammatory process and stop further nerve damage. It helps improve the symptoms too. Certain other immunosuppressant drugs are also used for treatment.

• In the case of relapsing chronic inflammatory demyelinating polyneuropathy, recovery depends on how severe the relapse was. Some patients may require hospitalization as they cannot walk and lose all mobility. Others with milder relapses may not need hospitalization, even though some of their abilities are lost. In the long term, it may cause residual problems like ongoing mild degrees of weakness and sensory loss. One may get fatigued fast with activities.

• Those recovering from chronic inflammatory demyelinating polyneuropathy should be supported with the following:

  • Physiotherapy and occupational therapy depend on the level of disability.

  • Psychotherapy and counseling.

  • Connecting with support groups for rehabilitation and awareness of better treatment options.

Conclusion:

It is important for the affected individual and family members of those diagnosed with chronic inflammatory demyelinating polyneuropathy to stay connected with support groups. It helps them stay aware of the treatment options and get access to expert neuromuscular physicians who has good knowledge of the disorder and the related complications. Though it is rare and difficult to diagnose, chronic inflammatory demyelinating polyneuropathy is treatable, and the patient can lead a relatively normal life afterward. Very rarely, one may even recover fully to attain normalcy. Hence, early diagnosis and treatment are key to bringing down the measure of functional disability related to chronic inflammatory demyelinating polyneuropathy.