Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
CIDP Symptoms | Causes
of CIDP | CIDP
Treatment | CIDP Cure | CIDP
Research
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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
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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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