WHAT is CIDP?
CIDP, or chronic inflammatory demyelinating polyneuropathy, is an immune-mediated neural disease that causes weakness and sensory loss in the limbs.

It is a rare disease, occurring in 1 in 100,000 people, and it occurs twice as often in men as in women. It also occurs more frequently in those with diabetes mellitus.
CIDP is often considered the chronic form of Guillain-Barrè syndrome (GBS) since they share many symptoms. Unlike GBS, which appears suddenly, CIDP tends either toward a chronic progression or a relapsing course.

WHAT ARE THE SYMPTOMS?
CIDP usually begins with tingling and numbness in the feet and spreads to the legs and hands. Often there is loss of sensation and dulled or absent reflexes. There may be weakness and fatigue. Because of numbness and weakness in the legs, some people have trouble walking and may trip and fall. Sometimes the symptoms of CIDP can mimic those of amyotrophic lateral sclerosis (ALS or Lou Gehrig’s disease.)

HOW IS IT DIAGNOSED?
There are several criteria that lead a doctor (usually a neurologist) to diagnose CIDP:

• 1. Progressive or relapsing sensory and motor nerve dysfunction of more than
      one limb, developing over at least 2 months.
• 2. Nerve conduction studies that show abnormalities.
• 3. Nerve biopsy showing damage to the myelin sheath, the fatty covering that
      protects the nerve.
• 4. Cerebral Spinal Fluid (CSF) tests that show slightly increased cell counts and
      increased protein concentration.

HOW IS CIDP TREATED?
There are three main treatments for CIDP:

• 1. IVIG: Intravenous immunoglobulin is an antibody treatment that is made from human blood. IVIG treatment usually begins over four or five days, and then follow-up doses are given a few days a month for several months. As symptoms improve, the dose and/or the frequency may be reduced. Most side effects are minor such as mild headaches and nausea.
• 2. Steroids: Treatment usually begins with intravenous methylprednisone over four or five days, and continues with daily high-dose oral prednisone. As symptoms improve, the dose may be gradually reduced. Side effects associated with long-term use of steroids include weight gain, diabetes, cataracts, and osteoporosis.
• 3. Plasma Exchange: This is a process that removes excess antibodies from the blood stream. The plasma exchange is usually performed 2 – 3 times per week for the first two or three weeks, followed by one exchange per week for the next 3 - 4 weeks. Side effects are infrequent and include dizziness and nausea. Access to veins may create a problem in some patients.

WHICH TREATMENT IS RIGHT FOR ME?
IVIG is becoming the drug of choice for treating CIDP because it is well-tolerated and effective. However, different people have better responses to some treatments than others. Most people find that one of the above treatments, alone or in combination, works for them.

WHAT IS THE PROGNOSIS?
The progression of CIDP varies from person to person, so it is difficult to give an overall prognosis. Starting treatment early helps prevent loss of nerve axons.
A small group of people experience a complete recovery, but some people will continue to have weakness and/or numbness. With ongoing treatment, however, most people are able to manage the disease and lead a normal life.

SUPPORT GROUPS
There are groups across the country that can provide information and support to those with CIDP. Keep in mind that CIDP is the chronic form of Guillain-Barrè syndrome (GBS), and various GBS groups may be useful as well.

CIDP International Foundation:
www.cidpusa.org

REFERENCES

• 1. Sharma KR et al. Demyelinating neuropathy in diabetes mellitus. Arch Neurol 2002 May;59(5):758-65.
• 2. Baba M. New trends in neuropathy practice: clinical approach to CIDP. Rinsho Shinkeigaku 2001 Dec;41(12):1210-3.