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Guillain-Barre syndrome

Definition:

Guillain-Barre syndrome is a serious disorder that occurs when the body's defense (immune) system mistakenly attacks part of the nervous system. This leads to nerve inflammation that causes muscle weakness .



Alternative Names:

Landry-Guillain-Barre syndrome; Acute idiopathic polyneuritis; Infectious polyneuritis; Acute inflammatory polyneuropathy; Acute inflammatory demyelinating polyneuropathy



Causes, incidence, and risk factors:

Guillain-Barre syndrome is an autoimmune disorder (the body's immune system attacks itself). Exactly what triggers Guillain-Barre syndrome is unknown. The syndrome may occur at any age, but is most common in people of both sexes between ages 30 and 50.

It often follows a minor infection, such as a lung infection or gastrointestinal infection. Most of the time, signs of the original infection have disappeared before the symptoms of Guillain-Barre begin.

The swine flu vaccination in 1976 may have caused rare cases of Guillain-Barre syndrome. However, the swine flu and the regular flu vaccines used today have not resulted in more cases of the illness.

Guillain-Barre syndrome damages parts of nerves. This nerve damage causes tingling, muscle weakness , and paralysis . Guillain-Barre syndrome most often affects the nerve's covering (myelin sheath). Such damage is called demyelination , and it causes nerve signals to move more slowly. Damage to other parts of the nerve can cause the nerve to stop working altogether.

Guillain-Barre syndrome may occur along with viral infections such as:

It may also occur with other medical conditions such as systemic lupus erythematosus or Hodgkin's disease .

Some people may get Guillain-Barre syndrome after a bacterial infection. A similar syndrome may occur after surgery, or when someone is critically ill (neuropathy of critical illness).



Symptoms:

Symptoms of Guillain-Barre can get worse very quickly. It may take only a few hours to reach the most severe symptoms, but weakness that increases over several days is also common.

Muscle weakness or the loss of muscle function (paralysis) affects both sides of the body. In most cases, the muscle weakness starts in the legs and then spreads to the arms. This is called ascending paralysis.

Patients may notice tingling, foot or hand pain, and clumsiness. If the inflammation affects the nerves to the diaphragm and chest and there is weakness in those muscles, the person may need breathing assistance.

Typical symptoms include:

  • Loss of reflexes in the arms and legs
  • Low blood pressure or poor blood pressure control
  • Muscle weakness or loss of muscle function (paralysis)
    • In mild cases, there may be weakness instead of paralysis
    • May begin in the arms and legs at the same time
    • May get worse over 24 to 72 hours
    • May occur in the nerves of the head only
    • May start in the arms and move downward
    • May start in the feet and legs and move up to the arms and head
  • Numbness
  • Sensation changes
  • Tenderness or muscle pain (may be a cramp-like pain)
  • Uncoordinated movement

Other symptoms may include:

  • Blurred vision
  • Clumsiness and falling
  • Difficulty moving face muscles
  • Muscle contractions
  • Palpitations (sensation of feeling the heart beat)

Emergency symptoms (seek immediate medical help):



Signs and tests:

A history of increasing muscle weakness and paralysis may be a sign of Guillain-Barre syndrome, especially if there was a recent illness.

A medical exam may show muscle weakness and problems with involuntary (autonomic) body functions, such as blood pressure and heart rate. The examination will also show that reflexes, such as the "ankle or knee jerk," are decreased or missing.

There may be signs of decreased breathing caused by paralysis of the breathing muscles.

The following tests may be ordered:



Treatment:

There is no cure for Guillain-Barre syndrome. However, many treatments are available to help reduce symptoms, treat complications, and speed up recovery.

When symptoms are severe, the patient will need to go to the hospital for treatment, which may include artificial breathing support.

In the early stages of the illness, treatments that remove or block the proteins that attack the nerve cells, called antibodies , may reduce the severity and duration of Guillain-Barre symptoms.

One method is called plasmapheresis, and it is used to remove the antibodies from the blood. The process involves taking blood from the body, usually from the arm, pumping it into a machine that removes the antibodies, and then sending it back into the body.

A second method is to block the antibodies using high-dose immunoglobulin therapy (IVIG). In this case, the immunoglobulins are added to the blood in large quantities, blocking the antibodies that cause inflammation.

Other treatments are directed at preventing complications.

  • Blood thinners may be used to prevent blood clots.
  • If the diaphragm is weak, breathing support or even a breathing tube and ventilator may be needed.
  • Pain is treated with anti-inflammatory medicines and narcotics, if needed.
  • Proper body positioning or a feeding tube may be used to prevent choking during feeding if the muscles used for swallowing are weak.


Support Groups:

Guillain-Barre Syndrome Foundation International - www.gbs-cidp.org



Expectations (prognosis):

Recovery can take weeks, months, or years. Most people survive and recover completely. According to the National Institute of Neurological Disorders and Stroke, about 30% of patients still have some weakness after 3 years. Mild weakness may persist for some people.

A patient's outcome is most likely to be very good when the symptoms go away within 3 weeks after they first started.



Complications:
  • Breathing difficulty (respiratory failure)
  • Contractures of joints or other deformity
  • Deep vein thrombosis (blood clots that form when someone is inactive or confined to bed)
  • Increased risk of infections
  • Low or unstable blood pressure
  • Paralysis that is permanent
  • Pneumonia
  • Skin damage (ulcers )
  • Sucking food or fluids into the lungs (aspiration )


Calling your health care provider:

Seek immediate medical help if you have any of the following symptoms:

  • Can't take a deep breath
  • Decreased feeling (sensation)
    • Difficulty breathing
    • Difficulty swallowing
    • Fainting
    • Loss of movement


Prevention:



References:

Hughes RA, Raphael JC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2009;(1):CD002063.

Hughes RA, Wijdicks EF, Barohn R, et al. Practice parameter: immunotherapy for Guillain-Barre syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003;61(6):736-740.

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




Review Date: 6/15/2010
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Daniel B. Hoch, PhD, MD, Assistant Professor of Neurology, Harvard Medical School, Department of Neurology, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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