Guillain-Barre syndrome

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

Definition
Guillain-Barre syndrome is a disorder caused by nerve inflammation involving progressive muscle weakness or paralysis, which often follows an infectious illness.

Causes, incidence, and risk factors

Guillain-Barre syndrome is an acute type of nerve inflammation. The inflammation damages portions of the nerve cell, resulting in muscle weakness or paralysis and sensory loss. The damage usually includes loss of the myelin sheath of the nerve (demyelination), which slows the conduction of impulses through the nerve.

The damage can also cause kill the axon part of the nerve cell (denervation), which stops nerve function entirely. Without the axon, messages cannot be transferred from one nerve cell to another.

The exact cause of this disorder is unknown. It may occur at any age but is most common in people of both sexes between the ages 30 and 50. It often follows a minor infection, usually a respiratory (lung) infection or gastrointestinal (gut) infection. Frequently, signs of the original infection have disappeared before the signs of Guillain-Barre begin.

Guillain-Barre syndrome may occur in association with viral infections like mononucleosis, AIDS, and herpes simplex, or after infections with bacteria (such as mycoplasma), and some types of diarrhea. Sometimes Guillain-Barre occurs following surgery or vaccinations (such as rabies and swine flu vaccines) or in association with other medical problems such as systemic lupus erythematosus or Hodgkin’s disease.

Symptoms

The disorder progresses rapidly (from days to a few weeks), causing weakness or paralysis equally on both sides of the body. As weakness starts in the legs and then spreads to the arms, it is referred to as ascending paralysis. At the same time, patients may notice tingling, foot or hand pain, and clumsiness. As paralysis worsens, the patient may require assisted ventilation in order to breathe.

The beginning phase of the illness is a rapid worsening that may take only a few hours to reach the most severe symptoms. These severe symptoms may last up to 3 weeks. This is followed by a plateau phase of no changes, then a recovery phase of improvement over days to 6 months or longer.

Typical symptoms include the following:

     
  • Muscle weakness or paralysis (the most common symptom) or uncoordinated movements:       o Weakness begins in the feet and legs and may progress upward to the arms and cranial (head) nerves       o May progress rapidly over 24 to 72 hours       o May begin in the arms and progress downward       o May occur in the arms and legs at the same time       o May occur in the cranial nerves only       o May not occur (mild cases)  
  • Sensation changes       o Numbness, decreased sensation       o Tenderness or muscle pain (may be a cramp-like pain)       o Usually accompanies or precedes muscle weakness       o May not occur at all

Additional symptoms that may be associated with this disease:

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

Symptoms indicating an emergency:

     
  • Difficulty swallowing  
  • Drooling  
  • Difficulty breathing  
  • Breathing, absent temporarily  
  • Inability to take a deep breath  
  • Fainting

Signs and tests

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

Neurological examination shows muscle weakness and may indicate dysfunction of the control of involuntary (autonomic) body functions such as blood pressure and heart rate. Sensory loss may be minimal, even when sensory changes are present.

There may be evidence of decreased breathing ability caused by paralysis of the breathing muscles, and a decrease in deep tendon reflexes in the arms and legs.

     
  • An NCV (nerve conduction velocity) shows demyelination.  
  • An EMG (a test of electrical activity in muscles) shows lack of nervous stimulation.  
  • A CSF (cerebrospinal fluid) examination may be abnormal, showing increase in protein without increase in white blood cell count.  
  • An ECG may show abnormalities in some cases.

Treatment
In the past, before treatment was available, many patients recovered completely with time. However, symptoms may be severe during the course of the disorder, and hospitalization is usually required to support breathing function and prevent complications and to provide physical therapy.

Plasmaphoresis may decrease the severity of the symptoms and facilitate a more rapid recovery. In this procedure, blood plasma, which contains antibodies, is removed from the body and replaced with intravenous fluids or antibody-free donated plasma. Intravenous immune globulin (IVIg) is equally effective in reducing the severity and duration of the symptoms.

Other treatments are directed at preventing complications such as choking during feeding (through positioning or use of a feeding tube), blood clots (through positioning and sometimes blood thinners), intermittent bladder catheterization, and others. Pain is treated aggressively with anti-inflammatory agents and narcotics if needed.

Support Groups

Guillain-Barre Syndrome Foundation International

Expectations (prognosis)
Almost all cases (95%) survive and the majority recover completely. Mild weakness may persist for some people. The outcome is most likely to be very good when symptoms remit within 3 weeks of their onset.

Complications

     
  • Breathing difficulty (respiratory failure)  
  • Aspiration of food or fluids into the lungs  
  • Pneumonia  
  • Increased risk of infections  
  • Deep vein thrombosis  
  • Permanent loss of movement of an area  
  • Contractures of joints or other deformity

Calling your health care provider
Go to the emergency room or call the local emergency number (such as 911) if loss of movement, decreased sensation or other emergency symptoms occur, including difficulty swallowing or breathing, feeling “unable to take a deep breath,” or fainting.

Johns Hopkins patient information

Last revised: December 4, 2012
by Amalia K. Gagarina, M.S., R.D.

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