Guillain-Barre syndrome

Answer 1

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

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, usually a lung infection or gastrointestinal infection. Usually, signs of the original infection have disappeared before the symptoms of Guillain-Barre begin.

Guillain-Barre syndrome causes inflammation that damages parts of nerves. This nerve damage causes tingling, muscle weakness, and paralysis. The inflammation usually affects the nerve's covering (myelin sheath). Such damage is called demyelination. Demyelination slows nerve signaling. Damage to other parts of the nerve can cause the nerve to stop working.

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

• AIDS
• Herpes simplex
• Mononucleosis

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 or certain vaccinations (such as rabies and Swine Flu). A similar syndrome may occur after surgery, or when critically ill.

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 increasing 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 there is weakness in those muscles, the person may need breathing assistance.

Typical symptoms include:

• Loss of reflexes in the arms and legs
• Muscle weakness or loss of muscle function (paralysis)
  • In mild cases, there may be no weakness or 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, decreased sensation
• Sensation changes
• Tenderness or muscle pain (may be a cramp-like pain)
• Uncoordinated movement

Additional symptoms may include:

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

Emergency symptoms (seek immediate medical help):

• Breathing temporarily stops
• Can't take a deep breath
• Difficulty breathing
• Difficulty swallowing
• Drooling
• Fainting
• Feeling light-headed when standing

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 may also show that reflexes, such as the "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:

• Cerebrospinal fluid sample ("spinal tap") may have increased levels of protein without an increase in white blood cells.
• ECG may show heart problems in some cases.
• EMG tests the electrical activity in muscles. It may show that nerves do not react properly to stimulation.
• Nerve conduction velocity test shows that electrical activity along the nerves is slowed or blocked.

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 breathing help, treatment, and physical therapy.

A method called plasmapheresis is used to remove proteins, called 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, then sending it back into the body.

High-dose immunoglobulin therapy (IVIg) is another treatment used to reduce the severity and length of Guillain-Barre symptoms. In this case, the immunoglobulins are added to the blood in large quantity, 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 week, breathing support or even a breathing tube and ventilator may be needed.
• Pain is treated aggressively 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 for swallowing are weak.

Support Groups

Guillain-Barre Syndrome Foundation International - www.gbsfi.com

Expectations (prognosis)

Recovery can take weeks 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
• Permanent loss of movement of an area
• Pneumonia
• 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

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.

Sharar E. Current therapeutic options in severe Guillain-Barre syndrome. Clin Neuropharmacol. 2006;29(1):45-51.

Roos KL. Viral infections. In: Goetz CG, ed. Textbook of Clinical Neurology. 3rd ed. Philadelphia, PA: Saunders Elsevier; 2007:chap 41.

Answer 2

The first symptoms to make themselves known are a tingling in the extremities (arms, hands, feet, and toes). Other symptoms include an inability to walk straight, high or low blood pressure, rapid heart rate, and difficulty with bladder function.

Answer 3

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:

• AIDS
• Herpes simplex
• Mononucleosis

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):

• Breathing temporarily stops
• Can't take a deep breath
• Difficulty breathing
• Difficulty swallowing
• Drooling
• Fainting
• Feeling light-headed when standing

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:

• Cerebrospinal fluid sample ("spinal tap")
• ECG
• Electromyography (EMG) tests the electrical activity in muscles
• Nerve conduction velocity test
• Pulmonary function tests

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

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.

Reviewed By

Review Date: 06/15/2010

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.

Answer 4

Muscle weakness in the legs occurs first, then the arms and face. Paresthesias (a prickly, tingling sensation) is also felt