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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

CIDP Symptoms | Causes of CIDP | CIDP Treatment | CIDP Cure | CIDP Research

Nerve conduction studies showing conduction block

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a form of nerve disease or peripheral neuropathy.  The disease can often be difficult to diagnose and can begin in many ways. Some patients experience numbness which spreads and becomes more intense over days to weeks. Other patients develop weakness which also progresses. In some cases the weakness can be so severe as to require hospitalization. The key feature is that the neurologic symptoms progresses over days to weeks.

CIDP Symptoms

The symptoms of CIDP begin with a combination of muscle weakness, as well as numbness and pain in the extremities. A person may also have impaired balance and difficulty walking as an early symptom. The disease usually begins in the legs but can begin in the arms at times.  The disease can affect people of any age from childhood through one’s 80’s. Once the disease begins it typically progresses and can cause severe weakness and even death if left untreated. The time to progress can vary from a matter of days to weeks to even months.  CIDP does not typically involve the muscles of the eyes or the heart. The diagnosis of CIDP is most commonly made by a neurologist. The tests required for the diagnosis usually involve EMG/NCS to study the way in which the nerves are functioning. A spinal tap if often performed and in some cases a muscle and nerve biopsy is required.  This disease seems to be more common in patients with diabetes and therefore anyone with diabetes who has symptoms of a neuropathy which are progressing quickly should be evaluated by  a neurologist with expertise in neuromuscular diseases.

CIDP Cause

Area of demyelination has had myelin stripped off
Area of demyelination has had myelin stripped off

CIDP is caused by a person’s immune system. The immune system is supposed to fight off bacteria and viruses, but in some people their immune system may start to attack their own body.  In CIDP the immune system starts to attack the insulation around the nerves, which is called myelin. Hence the neuropathy received the name demyelinating.  As the myelin around the nerves is damaged, the ability of the nerves to conduct electricity worsens. Frequently patients will recall a preceding viral infection a few weeks before the onset of their nerve disease.  In some cases we can identify specific antibodies in a patient’s blood which will allow us to classify the CIDP more specifically. We also believe that CIDP may be more common in certain other disease such as diabetes and some forms of cancer.

CIDP Treatment

The mainstay of therapy for CIDP is corticosteroids. While steroids have many side effects they are very effective in treating CIDP. Patients will often see an improvement in a matter of weeks. In patients who do not respond completely to steroids or who have severe side effects to steroids there are a number of steroid sparing drugs. These include mycophenylate, azathioprine, cyclosporine, or cyclophosphamide.  In addition patients often have a very dramatic improvement to therapy with intravenous gammaglobulin. In severe cases patients may also be treated through a process called plasmaphresis. This process removes blood, washes away antibodies and returns the blood back to the person, a process very much like dialysis.

CIDP can frequently follow a relapsing/remitting course. That is to say a patient may do very well for months or years and then for no clear reason experience a relapse. For this reason it is very important to follow closely with a neurologist who is familiar with the various treatments available for this disease.

Most patients (80%) make a total recovery but it may take weeks to years to see a maximal recovery. Some patients do not recover completely and have residual weakness, numbness and occasional pain. A small number are unable to resume their normal occupation. Death resulting from CIDP is highly unlikely, if recognized and treated approiately.

CIDP Cure

Once the process begins in CIDP there does not appear to be any way to cure it. There are however many form of medical therapy to treat the disease. The disease also may have periods of remission and then have periods of relapse but in most patients they will require some form of therapy for life.

CIDP Research

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