by Drs.Like Wu, Xiaojuan Wang, Bo Cheng, Susan Chu and Shuangshuang Liu
Wu Medical Center, Bejing, China
CIDP (Chronic Inflammatory Demyelinating Polyneuropathy) is a rare peripheral nerve disorder characterized by a gradual weakness in the legs and, to a lesser extent, the arms.
It is the gradual onset, as well as the chronic nature of CIDP, that differentiates it from GBS. Fortunately, CIDP is even rarer than GBS. The estimated amount of new cases is between 1.5 and 3.6 in a million people (compare to GBS: 1-2 in 100,000).
Like GBS, CIDP is caused by damage to the covering of the nerves, called myelin. It can start at any age and can occur in both genders. The weakness occurs over two or more months.
Unlike GBS, CIDP is not self-limiting (with an end to the acute phase). Left untreated, 30% of CIDP patients will reach the point that they are dependent on a wheelchair. Early recognition and treatment can avoid the more serious disabling effects of the disorder.
What causes CIDP?
Current theory holds that the body's immune system which normally protects itself, perceives myelin as a foreign object, and therefore attacks it. What actually starts this process is not clear. Some patients are found to have abnormal proteins in their blood, and these may cause damage.
How is CIDP diagnosed?
Diagnosis of CIDP is based on the symptoms experienced by the patient:
Difficulty walking, which progressively worsens over a few months
Tingling or other abnormal sensations may be experienced
A loss of reflexes, such as twitching in the knee and ankle
Tests may include:
An electrical test, which is a nerve conduction velocity-electromyography study
A spinal tap, to analyze cerebrospinal fluid
Blood and urine tests, including analysis of proteins
There are three general forms of CIDP:
Progressive form - extending over several years
Recurrent form - with multiple episodes that may be separated by months or years
Monophasic form - in which a single episode extends over one to three years without recurrence
How is CIDP treated?
Prednisone, similar to protective anti-inflammatory corticosteroids that are normally made by the body, may be used as an initial treatment for several reasons. It often improves strength, can conveniently be taken by mouth, and is inexpensive. Side effects can limit its use.
High dose Intravenous Immune Globulins (IVIG), protective blood proteins obtained from healthy volunteers, can be readily be injected into the vein in the patient's arm.
Plasma Exchange (PE), or Plasmapheresis, is when some of the patient's blood is removed and the blood cells returned without the liquid plasma portion of the patient's blood. It may work by removing harmful antibodies contained in the plasma.
2. Treatment for sequela
The chronic process of CIDP causes many patients to have nerve myelin sheath and axon damage, and permanent nerve dysfunction, such as paralysis, speech that is not fluent, dysphagia, urinary disorders, etc.
There have not been any specific treatments available for those with sequela in past few years. The Wu Stem Cell Medical Center (WSCMC) uses stem cells transplantation to treat many cases of CIPD, which has resulted in a clear recovery for many patients.
This medical center found that neural stem cells can be effective in repairing damage in the nerve myelin sheath and axon, and mesenchymal stem cells can be effective for immune regulation, reducing immune system attacks, and preventing recurrence of the disease. Combining these two kinds of stem cells provides a more effective treatment for CIDP patients.