Chronic inflammatory polyneuropathy

Definition

Chronic inflammatory polyneuropathy involves nerve swelling and irritation (inflammation) that leads to a loss of strength or sensation.

Alternative Names

Polyneuropathy - chronic inflammatory; CIDP; Chronic inflammatory demyelinating polyneuropathy

Causes, incidence, and risk factors

Chronic inflammatory polyneuropathy leads to a common type of damage to nerves outside the brain or spinal cord (peripheral neuropathy). Polyneuropathy means several nerves are involved. It usually affects both sides of the body the same amount.

The cause of chronic inflammatory polyneuropathy is an abnormal immune response. The specific triggers vary. In many cases, the cause cannot be identified.

It may occur with other conditions, such as:

• Chronic hepatitis
• Diabetes
• HIV
• Inflammatory bowel disease
• Systemic lupus erythematosus
• Lymphoma
• Thyrotoxicosis

Symptoms

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

Other symptoms that can occur with this disease:

• Abnormal movement
• Bowel or bladder problems
• Breathing difficulty
• Fatigue
• Hoarseness or changing voice
• Loss of function or feeling in the muscles
• Muscle atrophy
• Muscle contractions
• Paralysis of the face
• Speech impairment
• Swallowing difficulty
• Uncoordinated movement

Signs and tests

The doctor will examine you and ask questions about your medical history. The physical exam shows:

• Loss of muscle mass
• No reflexes
• Paralysis
• Muscle weakness
• Sensation problems on both sides of the body

Tests may include:

• Electromyography (EMG)
• Nerve conduction tests
• Nerve biopsy
• Spinal tap

Which other tests are done depends on the suspected cause of the condition, and may include x-rays, imaging scans, and blood tests.

Treatment

The goal of treatment is to control symptoms. What treatment is given depends on how severe the symptoms are, among other things. The most aggressive treatment is usually only given if you have difficulty walking or if symptoms interfere with your ability to care for yourself or perform work functions.

Treatments may include:

• Corticosteroids to help reduce inflammation and relieve symptoms
• Other medications that suppress the immune system (for some severe cases)
• Removing antibodies from the blood, using plasmapheresis or plasma exchange
• Intravenous immune globulin (IVIg), which involves adding large numbers of antibodies to the blood plasma to reduce the effect of the antibodies that are causing the problem

Support Groups

Expectations (prognosis)

The outcome varies. The disorder may continue long-term, or you may have repeated episodes of symptoms. Complete recovery is possible, but permanent loss of nerve function is not uncommon.

Complications

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

Calling your health care provider

Call your health care provider if you have a loss of movement or sensation in any area of the body, especially if your symptoms get worse.

Prevention

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References

Shy ME. Peripheral neuropathies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 446.