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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) 101

RamG Vallath and his motivational story

What is Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune neurological disorder in which there is inflammation of nerve roots and peripheral nerves and destruction of the fatty protective covering (myelin sheath) around the nerves. This affects how fast the nerve signals are transmitted and leads to loss of nerve fibers. This causes weakness, paralysis and/or impairment in motor function, especially of the arms and legs (limbs).The motor and sensory impairments usually affect both sides of the body (symmetrical) and the degree of severity and the course of disease may vary from case to case. Some affected individuals may follow a slow steady pattern of symptoms while others may have symptoms that stabilize and then relapse.

Anyone can get CIDP, but it is most common in older adults, and more in men than women. CIDP is not easy to diagnose. The condition affects 5–7 people in 100,000. As the condition progresses the patient may be required to use a wheelchair for mobility.

Research suggests that it might be an autoimmune disorder and the exact reason behind its cause is still unknown.

  • The most common symptoms of CIDP are weakness, numbness, and tingling in the legs, arms, fingers, and hands. Other symptoms include fatigue, pain, balance issues, and impairment of your ability to walk. Some people have described feeling as if there were an electrical storm in their arms or legs.
  • Symptoms usually occur on both sides of the body at the same time. But sometimes only one side is involved. You may experience symptoms in your head, arms, fingers and hands, legs, and toes and feet. Symptoms of CIDP can be mild, moderate or severe and change in severity over time.

There is no single confirmatory test to diagnosis CIDP. Instead, your doctor will ask you questions about your symptoms, such as when they started and how they feel. After a thorough clinical examination, other tests may be recommended.

A diagnosis of CIDP is usually reached after understanding electrophysiologic pattern of multifocal demyelination with tests like nerve conduction velocity studies, EMG, elevated CSF (cerebrospinal fluid) protein and nerve biopsies.

  • Corticosteroids – These medications bring down inflammation and slow the immune system. Steroids such as prednisone have proven effective in treating individuals with CIDP. However, individuals requiring high doses of corticosteroid drugs may experience side effects that deter long-term therapy
  • Intravenous immunoglobulin (IVIG) – Your doctor may give you injections of concentrated antibodies from healthy people to slow your body’s immune response
  • Plasma exchange (PE) – This treatment involves receiving a part of blood called plasma through an IV to slow down the immune system
  • Immunotherapy – These drugs interrupt the immune system to help stop it from attacking the myelin sheath
  • Stem cell therapy – In rare cases, the doctor may inject healthy stem cells (either the patient’s own or donated by someone else) to “reset” the immune system
Physiotherapy and exercises are recommended to keep the muscles and bones healthy.

The rate of progression is different for each patient and is difficult to predict. Some people go months or even years without a flare-up.

CIDP can become severely disabling. Some individuals may require mobility support with a wheelchair and other assistance for a day-to-day living.

There are potential therapies under investigation. There is a great deal of interest in using monoclonal antibodies to treat CIDP. Clinical trials are being developed to use rituximab, a monoclonal antibody against immune forming lymphocytes (B cells). Another monoclonal antibody under consideration is alemtuzumab which acts on both B cells and T cells providing a broader attack on the immune system. Some of the agents that have been found to be effective in multiple sclerosis are now being considered for CIDP. A clinical trial treating patients with fingolimod, a drug that affects the ability of lymphocytes to contribute to immune function is now underway.

Some organisations which raise awareness for CIDP are: GBS/CIDP Foundation International