Chronic Inflammatory Demyelinating Polyneuropathy

Chronic Inflammatory Demyelinating Polyneuropathy

Article
Brain & Nerve
Bone, Muscle, & Joint
+2
Contributed byMaulik P. Purohit MD MPHMar 04, 2020

What are the other Names for this Condition? (Also known as/Synonyms)

  • Chronic Relapsing Polyneuropathy
  • Lewis Summer Syndrome
  • Multi Focal Motor Neuropathy

What is Chronic Inflammatory Demyelinating Polyneuropathy? (Definition/Background Information)

  • Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an acquired disorder of the peripheral nerves that causes weakness and impaired sensations in the arms and legs
  • The condition results due to damage to the  myelin sheath, a fatty covering that wraps and protects the peripheral nerves
  • Corticosteroids and immunosuppressive drugs are the mainstay of treatment. Early treatment is recommended for CIDP to prevent further nerve damage, though remissions and relapses are common
  • Currently, there are no known preventive measures for Chronic Inflammatory Demyelinating Polyneuropathy

Who gets Chronic Inflammatory Demyelinating Polyneuropathy? (Age and Sex Distribution)

  • Chronic Inflammatory Demyelinating Polyneuropathy can occur at any age, but it is more common in young adults
  • The disorder is more common in men as compared to women
  • It is observed worldwide; no racial or ethnic preference is noted

What are the Risk Factors for Chronic Inflammatory Demyelinating Polyneuropathy? (Predisposing Factors)

The risk factors for Chronic Inflammatory Demyelinating Polyneuropathy may include:

  • A preceding infection
  • Male gender

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Chronic Inflammatory Demyelinating Polyneuropathy? (Etiology)

  • Chronic Inflammatory Demyelinating Polyneuropathy is an autoimmune disorder implying that the body’s own defense mechanism attacks the fatty protective covering of the nerves called myelin
  • In some individuals, abnormal proteins have been found to contribute to this damage

What are the Signs and Symptoms of Chronic Inflammatory Demyelinating Polyneuropathy?

The signs and symptoms associated with Chronic Inflammatory Demyelinating Polyneuropathy are:

  • Weakness  of  the arms and legs
  • Loss of sensation, such as tingling and numbness, usually beginning in one’s fingers and toes
  • Fatigue
  • Loss of deep tendon reflexes such as associated with knee and ankle jerk
  • The signs and symptoms of CIDP may be preceded by an infection.

How is Chronic Inflammatory Demyelinating Polyneuropathy Diagnosed?

Chronic Inflammatory Demyelinating Polyneuropathy may be diagnosed through:

  • Complete neurological exam and thorough evaluation of medical history
  • Cerebrospinal fluid (CSF) analysis
  • Blood tests that include complete blood counts (CBC), erythrocytic sedimentation rate (ESR), and anti-nuclear antibody (ANA) test
  • MRI spine with gadolinium enhancement
  • Electromyography (EMG)and nerve conduction studies (NCS) are used to differentiate peripheral neuropathy and demyelinating disease
  • Nerve biopsy for evaluation by a pathologist: A small sample of nerve tissue is examined under the microscope for further evaluation

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Chronic Inflammatory Demyelinating Polyneuropathy?

  • The complications of Chronic Inflammatory Demyelinating Polyneuropathy are:
  • Facial palsy: Disorder of the nerve that supplies the facial muscles resulting in their weakness
  • Double vision
  • Gait abnormality
  • Symmetric weakness of legs and arms
  • Some individuals may also have difficulty breathing, chewing, and swallowing
  • Cardiac arrest and respiratory failure may also develop

How is Chronic Inflammatory Demyelinating Polyneuropathy Treated?

Treatment of Chronic Inflammatory Demyelinating Polyneuropathy includes:

  • Corticosteroids and immunosuppressive drugs are the mainstay of treatment for CIDP
  • Plasmapheresis (plasma exchange therapy) and intravenous immunoglobulins are also effective
  • Physical and occupational therapy, exercises, and assistive devices may minimize deformity and improve muscles strength and function
  • Antiseizure medications, antidepressants, and pain medications may be required to treat pain and inflammation due to damaged nerves

How can Chronic Inflammatory Demyelinating Polyneuropathy be Prevented?

There are no preventive measures available for Chronic Inflammatory Demyelinating Polyneuropathy at present.

What is the Prognosis of Chronic Inflammatory Demyelinating Polyneuropathy? (Outcomes/Resolutions)

  • In order to prevent further nerve damage, an early treatment of Chronic Inflammatory Demyelinating Polyneuropathy is recommended
  • The prognosis of CIDP remains variable from spontaneous recovery to remissions and relapses
  • The condition may be monophasic, lasting for 1-3 years, or it may be recurrent or progressive in nature

Additional and Relevant Useful Information for Chronic Inflammatory Demyelinating Polyneuropathy:

Intravenous immunoglobulin (IVIG) is well tolerated in young adults. Plasma exchange is more useful for elderly adults and those with complications.

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Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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