CIDP - Chronic Inflammatory Demyelinating Polyneuropathy
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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an autoimmune disease where the body's immune system attacks its own nerves. During infancy the body's immune system learns to differentiate between self and non-self. This means that normally the body learns not to attack itself; It also knows when a foreign object like a virus or a bacteria has entered the body which needs to be destroyed. In autoimmune diseases, the recognition of self is lost and the body attacks itself. The nerves of the body are seen as a foreign object and attacked. If this occurs acutely then the illness is called Guillain-Barré and if it becomes chronic then it is called Chronic Inflammatory Demyelinating Polyneuropathy or CIDP.
A nerve is very similar to a wire. Just like a wire, the nerves work with electrical impulses. In a wire there is the copper part that transmits the electrical signal and there is also a plastic insulation around the copper wire. The nerve itself acts like the copper wire, and there is an insulation around the wire that is called myelin. In CIDP, it is the myelin that is affected first and in later stages of the disease the nerve itself is attacked. If we started treatment in the early stages were only the myelin is damaged, then the chances of recovery are much better as the myelin has very good regenerative properties.
The symptoms of CIDP are caused as a result of nerve injury. Although it could be a disabling condition, CIDP is a treatable condition. In order to provide effective treatment it is crucial to make the proper diagnosis, which could sometimes be difficult to make. We use the most advanced techniques to make the proper diagnose. Since the cause of the disease is immune mediated, to treat CIDP we use medications that modify the immune system. We could use a single treatment modality or a combination of modalities in the more difficult cases. Once we are able to stop the immune system's attack on the nerves, then the nerves are allowed to regenerate. With proper diagnosis and using the proper treatment modalities we have great success in the treatment of the most difficult cases.
The symptoms of Chronic Inflammatory Demyelinating Polyneuropathy include numbness, tingling, burning and weakness, generally occurring in the feet first and they gradually ascend to involve other parts of the body. One of the first signs that could develop is a foot drop, where patients develope weakness in their ankles and as a result tend to trip over their feet, or they could develop numbness and tingling in the feet and toes which gradually spreads to to the other parts of the body. Other organs could also become involved including inability to adequately empty the bladder, nausea, vomiting, abdominal fullness or bloating, diarrhea, or constipation, low blood with dizziness, or trouble maintaining an erection. The severity and the combination of symptoms vary. Some could have only numbness and tingling only, others could have weakness only. Some patients are severely affected and they could become completely paralyzed and have severe numbness and tingling. Others have a very slow progression with mild disease. These patients often complain of difficulty with walking. They develop poor balance and cannot walk for long periods of time.
The syndromes of CIDP are as follows:
At times, related physical symptoms also include:
Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy is based on the symptoms and clinical exam, from which we get an idea if the peripheral nerves are involved and if there is a neuropathy. To confirm neuropathy, and to decide whether or not the patient has CIDP, we send specialized laboratory tests and perform a nerve and muscle test called EMG nerve conduction study. In the nerve conduction study the nerves are stimulated, and based on their response we are able to tell if the nerve is damaged. In the second part of the test, the EMG, each muscle is tested individually and we are able to tell if the muscle or the nerve that controls that muscle is damaged. Based on this test we are able to tell whether the nerve or the muscles are injured, where in the body is the injury occurring, and which part of the nerve is not functioning properly. In addition we send specialized laboratory testing which helps us determine what the cause of injury is. In addition, at times, a spinal tap is done to analyze the spinal fluid for signs of CIDP. A muscle biopsy might be done in some cases to help with making the diagnosis.
Proper diagnosis is crucial since proper treatment could only be given if the diagnosis is correct. Dr. Shaoulian is an expert in diagnosing neuropathies, including CIDP, and in the performance of EMG nerve conduction studies.
Tests for diagnosis:
Laboratory Studies include:
Chronic Inflammatory Demyelinating Polyneuropathy has become one of the most treatable forms of neuropathy. There are currently multiple modalities that we are able to use for the treatment of CIDP. Since this is an immune mediated neuropathy, where the body is attacking the nerves, treatment of CIDP is aimed at modulating the immune system to stop the attack. Once the immune system stops attacking the nerves, the nerves could start to regenerate and reverse the symptoms of CIDP. One category of treatment options include immunosuppressant such as cytoxan, Imuran, and CellCept. These medications weaken the immune system so that it doesn't attack the nerves. Another potent treatment option is IVIG which is a pull of antibodies from more than a thousand patients which is infused to the patient. These antibodies protect the nerves of the body from being attacked. The mechanism is complex and is thought to involve multiple pathways. The next option is plasmapharesis. In this process the patient's blood is filtered and the antibodies and proteins that are responsible for attacking the nerves are removed from the blood.
In most patients we are able to see improvement using these treatment options alone or in combination. In patients in whom these treatment options are not effective, we are using newer treatments. We are now able to detect certain antibodies that attack the nerves and we have medications that could knock them out. When used properly, these treatment options are very effective with minimal side effects. In most patients we are able to significantly improve their symptoms even in the most difficult and severe cases. If you have been diagnosed with CIDP, and you are not happy with the results you are getting or you have been told that there is nothing else that can be done, we could help.