Karla News

What is CIDP?

Guillain Barre Syndrome, Ivig

Brooke’s friends often reminded her of how lucky she was. The 17-year-old high school junior had a body made for running. Her high school coach told her it wasn’t a question of if she would get a college scholarship but of how much she would be awarded. Neither of them had any idea that within a few months, she would be diagnosed with a condition known as CIDP.

Overview of CIDP

According to WebMD, CIDP is the abbreviation for a rare neurological condition with a very long name: chronic inflammatory demyelinating polyneuropathy. The illness is also known as chronic relapsing polyneuropathy but is usually referred to simply as CIDP.

A CIDP patient suffers from inflammation of nerve roots and peripheral nerves. The myelin sheath, a fatty covering over the nerves, has somehow been damaged.

CIDP is closely so closely related to Guillain-Barre syndrome that doctors consider it the chronic version of that illness, the National Institute of Neurological Disorders and Stroke (NINDS) reports. Experts associate its occurrence with some type of defect in the patient’s immune system that causes it to consider myelin as a foreign body and then attack and destroy it, according to GBS/CIDP Foundation International.

Signs and Symptoms of CIDP

This disorder can strike individuals of any age and gender but appears to be most prevalent in young adults and occurs more often in men than in women. The patient typically experiences slowly increasing weakness in the legs. The arms are less affected. To be considered CIDP, the condition must persist at least two months.

Many patients first report a tingling or numbness that starts in their fingers and toes. They eventually complain of generalized weakness in their arms and legs and lose their deep-tendon reflexes. Some experience abnormal sensations. Fatigue is common.

See also  Common Variable Immune Deficiency (CVID): Symptoms, Diagnosis and Treatment

Brooke began to have difficulty with her runs around the school track. She tired easily and felt that she somehow was no longer in control of her movements. When the doctor examined her, he immediately noticed that she had already lost the knee-jerk reflex in one leg. By that time, the teenager and her parents were understandably frightened that she would end up confined to a wheelchair.

Diagnosis and Treatment

Most patients, having no idea of what could be causing their symptoms, initially visit a pediatrician, family physician or internist. They are normally referred to a neurologist for a physical exam and a test known as a nerve conduction velocity-electromyography study.This procedure will reveal the problems with the conduction of electrical signals associated with CIDP.

If the results of this test are abnormal, doctors sometimes perform a spinal tap to analyze cerebrospinal fluid. In CIDP patients, it usually shows elevated protein levels with normal cells. However, some patients with one of the variants of CIDP show only the symptoms associated with the nerve conduction study. Laboratory tests on blood and urine samples are also standard.

Fortunately, though her condition was quickly deteriorating, there were several treatment options for Brooke. The most common is the use of corticosteroids like prednisone. Doctors might opt to try the steroid alone or with certain immunosuppressant medications. However, the side effects can be dramatic as well as serious.

Some CIDP patients respond extremely well to intravenous immunoglobulin (IVIg) therapy, in which they receive blood proteins from healthy donors. A third alternative – the one that eventually put Brooke back on the track – is plasmaphersis, also known as plasma exchange. During this procedure, doctors remove some of the individual’s blood. The blood cells are returned to the patient without the plasma that was in the blood.

See also  Living Through ITP

The prognosis of a patient with CIDP varies greatly. While some recover spontaneously, others suffer from many recurrences with what feels like some degree of recovery between them. However, the disorder is definitely considered treatable.

It isn’t uncommon for CIDP patients to require a series of different types of treatment over the course of their illness as the success rate begins to wane with each one. The earlier the treatment, the less likely the patient is to experience loss of nerve axons and the more likely he or she is to preserve mobility.

Sources:

http://www.webmd.com/brain/chronic-inflammatory-demyelinating-polyneuropathy

http://www.ninds.nih.gov/disorders/cidp/cidp.htm

http://www.gbs-cidp.org/aboutcidp.htm