Knowledge Base

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

What is Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder characterized by progressive weakness and impaired sensory function in the legs and arms. The disorder, which is sometimes called chronic relapsing polyneuropathy, is caused by damage to the myelin sheath (the fatty covering that wraps around and protects nerve fibers) of the peripheral nerves. CIDP is closely related to Guillain-Barre syndrome and it is considered the chronic counterpart of that acute disease.

What are the symptoms?

Although it can occur at any age and in both genders, CIDP is more common in young adults, and in men more so than women. It often presents with symptoms that include tingling or numbness (beginning in the toes and fingers), weakness of the arms and legs, loss of deep tendon reflexes (areflexia), fatigue, and abnormal sensations. 

Is there any treatment?

Treatment for CIDP includes corticosteroids such as prednisone, which may be prescribed alone or in combination with immunosuppressant drugs. Plasmapheresis (plasma exchange) and intravenous immunoglobulin (IVIg) therapy are effective. IVIg may be used even as a first-line therapy. Physiotherapy may improve muscle strength, function and mobility, and minimize the shrinkage of muscles and tendons and distortions of the joints.

What is the prognosis?

The course of CIDP varies widely among individuals. Some may have a bout of CIDP followed by spontaneous recovery, while others may have many bouts with partial recovery in between relapses. The disease is a treatable cause of acquired neuropathy and initiation of early treatment to prevent loss of nerve axons is recommended. However, some individuals are left with some residual numbness or weakness.

Related evidence

Klehmet J, Goehler J, Ulm L, Kohler S, Meisel C, Meisel A, Harms H.  Effective treatment with intravenous immunoglobulins reduces autoreactive T-cell response in patients with CIDP. J Neurol Neurosurg Psychiatry. 2014 Jul 29. pii: jnnp-2014-307708. doi: 10.1136/jnnp-2014-307708. [Epub ahead of print]

Sung JY, Tani JP, Park SB Kiernan MC, Lin CS.Early identification of 'acute-onset' chronic inflammatory demyelinating polyneuropathy. Brain. 2014 Aug;137(Pt 8):2155-63. doi: 10.1093/brain/awu158. Epub 2014 Jun 19.

Latov N. Diagnosis and treatment of chronic acquired demyelinating polyneuropathies.
 Nat Rev Neurol. 2014 Aug;10(8):435-46. doi: 10.1038/nrneurol.2014.117. Epub 2014 Jul 1.

References

Adapted with permission from the National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health, Bethesda, MD 20892, USA.

Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

Last updated August 5, 2014



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