Q: I’m a female, age 50. After three plus years of symptoms, I have been diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) this year by my neurologist. I have just completed my second month of intravenous immunoglobulin treatments. My PCP told me if I didn’t have these treatments, I would end up in a nursing home. The only other treatment available is to suppress my immune system but that is not an option since the risks of liver and/or brain cancer are too high.
Do you think I have any other options available and what can you tell me about CIDP? I could not come up with a cause, nor could my neurologist so any information you can provide would be appreciated.
A: CIDP is a neurological disorder that presents with progressive weakness and impaired sensory function of the extremities. Symptoms may include numbness and tingling that begins in the fingers and toes, areflexia (loss of deep tendon reflexes), fatigue, and weakness of the arms and legs. The disorder is caused by damage to the myelin sheath of the peripheral nerves. Myelin is the fatty covering that surrounds and protects nerve fibers. It has been noted that patients frequently report a viral infection several weeks prior to the onset of the disease. While both genders can be affected, CIDP is more common in men than in women and is seen in young adults more frequently than in older ones.
Diagnosis of CIDP is generally accomplished by an EMG so the neurologist can study the patient’s nerve function. In some instances, a spinal tap may be performed, while in other cases, a muscle and nerve biopsy may be done. CIDP is more common in those individuals with a diagnosis of diabetes.
The mainstay of therapy is steroids, plasma exchange and intravenous immunoglobulin therapy. Some physicians may choose an addition of imunosuppressant drugs. While steroids are known to have serious side effects, patients may find improvement in a matter of weeks once the therapy is begun. When patients fail to respond completely to steroids, there are also steroid sparing drugs that can be attempted. Plasma exchange and IV immunoglobulin therapy have also been found effective and physiotherapy may help improve muscle strength and mobility, while minimizing muscle and tendon shrinkage. Plasma exchange involves removing blood from the body, clearing away the antibodies, and returning the blood back to the individual in a process that resembles dialysis.
As I have said many times, disorders present differently and the response is different from patient to patient. Some individuals may experience spontaneous recovery, while others may only experience partial recovery between relapses. In some instances, an overall weakness may occur and be so severe as to require actual hospitalization. Generally speaking, symptoms may begin with muscle weakness, numbness, extremity pain, and a gait abnormality. Progression of symptoms may vary from days to weeks to months. One study from 2006 indicated complete remission (defined as lasting more than two years with normal nerve conduction studies), was noted in 26% of the patients observed. Partial remission (defined as being able to walk) was noted in 61% in the group and a severe disability (indicating an inability to walk) or relapses related to tapering of medication were present in 13%. Because of the periods of remission and relapses that occur, most patients will likely require some form of therapy for life.
