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Chronic Inflammatory Demyelinating Polyneuropathy

By Michael Rubin, MDCM

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

As in Guillain-Barré syndrome (see Guillain-Barré Syndrome), nerves are damaged by an attack by the body's immune system. Symptoms are similar. Weakness and abnormal sensations (numbness and a pins-and-needles sensation) progress for more than 8 weeks. Weakness can worsen continually or come and go. 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 (see Tests for Brain, Spinal Cord, and Nerve Disorders : Electromyography and Nerve Conduction Studies), and a spinal tap (lumbar puncture—see Figure: How a Spinal Tap Is Done) 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

  • Sometimes plasma exchange or immune globulin

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 or immune globulin given intravenously may be preferred to corticosteroids.

People may need treatment for months or years.

Drugs Mentioned In This Article

  • Generic Name
    Select Brand Names
  • RAYOS
  • IMURAN

* This is the Consumer Version. *