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What’s CIDP?

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder that causes damage to nerves in the peripheral nervous system. It is thought to occur when the immune system attacks the myelin sheath surrounding axons on neurons. Symptoms include muscle weakness, abnormal sensation, pain, and tingling. Treatment involves taming the immune system with corticosteroids, immunosuppressants, plasmapheresis, and intravenous immunoglobulin. Physical therapy can help regain lost muscle function, but some sensation and muscle loss may be permanent.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder that causes damage to nerves in the peripheral nervous system or to nerves outside the brain and spinal cord. Often, the cause of this disorder cannot be determined, although it has been associated with HIV, chronic active hepatitis, lupus erythematosus, blood cell disorders, and inflammatory bowel disease. The most common symptoms of chronic inflammatory demyelinating polyneuropathy include muscle fatigue, abnormal sensation, pain, or tingling, progressive loss of reflexes, muscle weakness, and possibly flaccid paralysis, especially in the arms or legs.

Chronic inflammatory demyelinating polyneuropathy is thought to occur when the immune system fails to recognize peripheral nerves and begins attacking them. Specifically, immune cells attack the myelin sheath. Myelin is a fatty coating that surrounds axons on neurons and helps conduct nerve impulses rapidly from neuron to neuron. When this is taken away, or the neurons are demyelinated, it causes the nerves to respond weakly or not at all. Because the disorder affects several nerves, it is called polyneuropathy and its chronic or gradual nature differentiates it from related acute diseases such as Guillan Barré syndrome.

CIDP is quite rare. It tends to affect men more than women and is more common in young adults. Although some people with CIDP may be able to wait and let symptoms go away on their own, most people with CIDP will never get symptom relief without treatment.

Symptoms of chronic inflammatory demyelinating polyneuropathy typically begin with a gradual weakening and loss or change of sensation in the arms and legs. Symptoms can progress to include facial weakness, trouble walking, problems with bowel and bladder control, joint pain, muscle wasting, trouble swallowing, trouble controlling movements, trouble breathing, facial paralysis, and hoarseness. A doctor might conduct an electromyography (EMG) exam, a nerve conduction velocity (NCV) test, a nerve biopsy, a spinal tap, and an X-ray to determine if CIDP is the cause of these symptoms.

Treatments for chronic inflammatory demyelinating polyneuropathy are usually concerned with taming the immune system. Neurologists and immunologists often prescribe corticosteroids and other immunosuppressants to keep the immune response at bay. Plasmapheresis is another common treatment. During this procedure, doctors remove plasma from the body, remove immune proteins called antibodies from the plasma, and then return the plasma to the body. Doctors can also use intravenous immunoglobulin (IVIG), a donated plasma product delivered into the bloodstream, to stop the immune system from causing inflammation where it shouldn’t. Physical therapy can help regain lost muscle function, but for many CIDP patients, some sensation and muscle loss will be permanent.

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