Not Found
Locations

Find information on medical topics, symptoms, drugs, procedures, news and more, written in everyday language.

* This is the Consumer Version. *

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

(Chronic Acquired Demyelinating Polyneuropathy; Chronic Relapsing Polyneuropathy)

By Michael Rubin, MDCM, Professor of Clinical Neurology;Attending Neurologist and Director, Neuromuscular Service and EMG Laboratory, Weill Cornell Medical College;New York Presbyterian Hospital-Cornell Medical Center

Chronic inflammatory demyelinating polyneuropathy is a form of polyneuropathy that, like Guillain-Barré syndrome, causes increasing muscle weakness, but the weakness progresses for more than 8 weeks.

  • Chronic inflammatory demyelinating polyneuropathy is thought to be caused by an autoimmune reaction that damages the myelin sheath around nerves.

  • In this disorder, weakness worsens continually over a period of more than 8 weeks.

  • Electromyography, nerve conduction studies, and analysis of cerebrospinal fluid can help confirm the diagnosis.

  • Treatment may include corticosteroids, drugs that inhibit the immune system, and sometimes immune globulin and plasma exchange.

Chronic inflammatory demyelinating polyneuropathy develops in 3 to 10% of people with Guillain-Barré syndrome.

As in Guillain-Barré syndrome, an autoimmune reaction, is thought to be involved. The body's immune system attacks the myelin sheath, which surrounds the nerve and enables nerve impulses to travel quickly.

Insulating a Nerve Fiber

Most nerve fibers inside and outside the brain are wrapped with many layers of tissue composed of a fat (lipoprotein) called myelin. These layers form the myelin sheath. Much like the insulation around an electrical wire, the myelin sheath enables electrical impulses to be conducted along the nerve fiber rapidly.

When the myelin sheath is damaged, nerves do not conduct electrical impulses normally.

Symptoms

Symptoms of chronic inflammatory demyelinating polyneuropathy are similar to those of Guillain-Barré syndrome: Weakness and abnormal sensations (numbness and a pins-and-needles sensation). However, these symptoms worsen for more than 8 weeks. (In Guillain-Barré syndrome, weakness usually worsens over 3 or 4 weeks, then remains the same or starts to return to normal.)

Reflexes are usually absent.

In most people with this disorder, blood pressure fluctuates less, abnormal heart rhythms occur less often, and other internal functions are less impaired than in people with Guillain-Barré syndrome. Also, weakness may be more irregular, affecting the two sides of the body differently, and weakness may progress more slowly.

Diagnosis

  • Electromyography, nerve conduction studies, and a spinal tap

Doctors suspect chronic inflammatory demyelinating polyneuropathy based on symptoms. It can be distinguished from Guillain-Barré syndrome because it progresses for more than 8 weeks.

Electromyography, nerve conduction studies, and a spinal tap (lumbar puncture) to obtain cerebrospinal fluid (which surrounds the brain and spinal cord) are done to confirm the diagnosis.

Rarely, a biopsy of the nerve is needed.

Treatment

  • Corticosteroids and/or drugs that inhibit the immune system

  • Immune globulin and plasma exchange

Corticosteroids such as prednisone can relieve symptoms in some people. Drugs that inhibit the immune system (immunosuppressants) such as azathioprine may also be used.

However, if chronic inflammatory demyelinating polyneuropathy is severe or progresses rapidly, plasma exchange (filtering of toxic substances, including antibodies to the myelin sheath, from the blood) or immune globulin (a solution containing many different antibodies collected from a group of donors) given intravenously are used. These treatments are often preferred to corticosteroids because corticosteroids have many side effects.

People may need treatment for months or years.

Resources In This Article

Drugs Mentioned In This Article

  • Generic Name
    Select Brand Names
  • IMURAN
  • RAYOS

* This is the Consumer Version. *