Chronic inflammatory polyneuropathy

Alternative names
Polyneuropathy - chronic inflammatory

Definition
Chronic inflammatory polyneuropathy is a disorder involving slowly progressive or repeated episodes of loss of movement or sensation, caused by inflammation of multiple nerves.

Causes, incidence, and risk factors

Chronic inflammatory polyneuropathy is a common type of damage to nerves outside the brain or spinal cord (peripheral neuropathy) involving multiple nerves (polyneuropathy). An acute form of the disorder is called Guillain-Barre syndrome.

Polyneuropathy implies a widespread process that usually affects both sides of the body equally. The cause of chronic inflammatory polyneuropathy is an abnormal immune response. The specific antigens, immune processes, and triggering factors are variable and in many cases are unknown.

It may occur in association with other conditions such as HIV, inflammatory bowel disease, lupus erythematosis, chronic active hepatitis, and blood cell abnormalities.

Symptoms

     
  • Weakness, usually in the arms and hands or legs and feet  
  • Facial weakness  
  • Difficulty walking  
  • Difficulty using the arms and hands or legs and feet  
  • Sensation changes (usually of the arms and hands or legs and feet)       o Pain, burning, tingling, or other abnormal sensations       o Numbness or decreased sensation

Additional symptoms that may be associated with this disease:

     
  • Swallowing difficulty  
  • Speech impairment  
  • Loss of function or feeling in the muscles  
  • Muscle contractions  
  • Muscle atrophy  
  • Uncoordinated movement  
  • Dysfunctional movement  
  • Joint pain  
  • Hoarseness or changing voice  
  • Fatigue  
  • Facial paralysis  
  • Bowel or bladder dysfunction  
  • Breathing difficulty

Signs and tests

A neurological examination shows sensory abnormalities and weakness. Sensation deficits usually occur in a symmetrical pattern, and progress from the extremities to central locations.

Reflexes are usually absent. Muscle weakness or paralysis of the affected area may be present, and muscle atrophy (loss of muscle mass) may be apparent. Nerves may be physically palpable. A nerve biopsy may be necessary to demonstrate an inflammatory cause and to rule out other nerve diseases.

A spinal tap may show elevated CSF total protein or other abnormalities that indicate an inflammatory neurologic process. EMG and nerve conduction tests may indicate loss of the myelin sheath (slow conduction rate) or degeneration of the axon of the nerve cell (normal conduction rate).

The selection of other tests is guided by the suspected cause of the disorder, as suggested by the history, symptoms and pattern of symptom development. The tests may include various X-rays, scans, and blood tests.

Treatment
The goal of treatment is control of symptoms. Treatment is based on severity and extent of symptoms, response to early treatment, and other factors. In general, aggressive treatment is reserved for instances where there is difficulty walking or if symptoms interfere with the ability to perform self-care or occupational functions.

Corticosteroids may be beneficial to reduce inflammation and alleviate symptoms. Other medications that suppress the immune system may also be used for some severe cases.

Plasmapheresis may reduce the immune response and may benefit some patients. In this procedure, blood plasma (the fluid portion) containing antibodies is removed and replaced with intravenous fluids or donated plasma that is antibody-free. Intravenous immune globulin (IVIg) is another effective option.

Expectations (prognosis)
The outcome varies. The disorder may be prolonged, or there may be repeated episodes of symptoms. Complete recovery is possible, but permanent loss of nerve function may also occur.

Complications

     
  • Permanent weakness or paralysis of areas of the body  
  • Permanent decrease or loss of sensation in areas of the body  
  • Repeated or unnoticed injury to an area of the body  
  • Side effects of medications used to treat the disorder (see the specific medication)  
  • Pain

Calling your health care provider
Call your health care provider if loss of movement or sensation in any area of the body occurs. Progressive symptoms are particularly worrisome.

Johns Hopkins patient information

Last revised: December 5, 2012
by David A. Scott, M.D.

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