Guillain-Barre syndrome is a rare but serious autoimmune disease involving the nervous system. It affects around 1 in 100,000 people, or 3,000-6,000 Americans yearly.1
The disorder is characterized by an assault on the peripheral nervous system leading to weakness and paralysis that can last months or years. It frequently follows a mild viral infection which may be flu-like, or even a gastroenteritis, with symptoms starting 10-14 days after the infection.
Both sexes can develop Guillain-Barre syndrome and it can strike at any age, although it is slightly more common in older male adults. The precise cause is not known but the condition usually begins shortly after an individual has experienced an infectious disease.
In this article, we will look at the symptoms, causes, diagnosis and treatment of Guillain-Barre syndrome. We will also investigate the connections between the Zika virus, vaccinations and Guillain-Barre.
Contents of this article:
Here are some key points about Guillain-Barre syndrome. More detail and supporting information is in the main article.
- Guillain-Barre syndrome is an autoimmune disease
- Autoimmune diseases are where an individual's immune system destroys healthy tissue
- The initial symptoms of Guillain-Barre syndrome are tingling and muscle weakness
- Eventually, the entire body can become paralyzed
- The condition was first described in 1859 by the French physician Jean Landry
- The exact causes of Guillain-Barre syndrome are still unknown
- Symptoms tend to develop very rapidly, over a small number of days
- There is a rare variant of Guillain-Barre syndrome called Miller Fisher syndrome that predominantly affects the muscles of the face and eye movements
- The onset of Guillain-Barre syndrome is often preceded by an infection
- Most patients fully recover within 12 months.
What is Guillain-Barre syndrome?
Guillain-Barre syndrome affects the myelin coating of peripheral nerves.
Guillain-Barre syndrome is a rare but serious autoimmune disorder that affects the peripheral nervous system (any part of the nervous system outside of the brain and spinal cord).
An autoimmune disease involves the patient's immune system attacking and destroying certain groups of healthy cells.
In the case of Guillain-Barre syndrome, it is the myelin sheaths of peripheral nerves that are the targets of the autoimmune response.
The nerves' myelin sheaths are essential for carrying nerve impulses.
In some cases, the nerve axons are also attacked - these are the long, thin extensions of nerve cells.
As the myelin is slowly removed, nerves can no longer transmit information such as touch sensations to the brain, hence the numbness. In addition, the brain is no longer able to transmit signals back to the body; this explains the muscle weakness.
The disease normally begins as a tingling sensation and weakness in the extremities - often the feet and legs - slowly spreading until a large portion of the body is affected. This is because the nerves running to the lower extremities are the longest in the body and, therefore, easier to attack and compromise.
Guillain-Barre syndrome is considered an emergency and should be treated by medical professionals as soon as possible.
To date, the exact causes of Guillain-Barre syndrome are not known, but the disease often follows soon after other illnesses have occurred.
There is currently no cure for Guillain-Barre syndrome, but there are medications available to ease symptoms and the majority of patients make a full recovery. Others may have lingering, minor problems such as tingling or weakness in some areas of the body.
Initially, Guillain-Barre syndrome was considered to be one single condition. Now, it is considered to occur in a number of forms. The three most common forms of Guillain-Barre syndrome are as follows:2
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP): the most common variant in the US - weakness begins in the lower part of the body and gradually extends
- Miller Fisher syndrome (MFS): occurs in around 5% of American Guillain-Barre syndrome cases (it is more prevalent in Asian patients) - paralysis begins with the eyes and problems with walking are more common
- Acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN): these are the most rare versions of Guillain-Barre syndrome in the US, but more common in Japan, China and Mexico.
Symptoms of Guillain-Barre syndrome
Severe nerve pain due to the loss of myelin sheath is a common symptom of Guillain-Barre syndrome.
The symptoms of Guillain-Barre syndrome typically begin with tingling and weakness in the feet and legs that gradually moves up to the rest of the body. It is described as a "symmetrical ascending radiculopathy," meaning that often weakness starts in both legs and then slowly moves up the body with both arms being affected too.
Around 1 in 10 patients report the tingling beginning in their face or neck.
The weakness gradually worsens and turns into paralysis.
Symptoms and other complications of Guillain-Barre syndrome include:
- Weakness in lower body, moving upward
- General instability when walking
- Less control over facial muscles - when chewing or talking, for instance
- Cramp-like pain, worsening at night
- Lack of control over bowel or bladder
- Pain - around 50% of patients experience severe nerve pain that needs to be managed with medication
- Tachycardia - faster than normal heart rate - this is a common symptom
- Particularly high or low blood pressure
- Blood clots - the associated immobility produced by Guillain-Barre syndrome increases the risk of clots. Blood thinning agents and support socks may be recommended
- Pressure sores may develop for the same reasons as stated above
- Difficulty breathing - 30% of patients will need temporary help with breathing
- Relapse - up to 5% of patients with Guillain-Barre syndrome will have some degree of relapse at some point in the future
- Psychological difficulties may occur due to the rapid onset of paralysis and the reliance on others to help complete basic everyday tasks.
Dr. Helen Webberley explained to Medical News Today what one of the major worries about Guillain-Barre syndrome is:
The great concern is that the muscles responsible for our breathing may also be affected so that victims simply cannot breathe without assistance and that means being hooked up to a ventilator in an intensive care unit. An "outbreak" of Guillain-Barre syndrome is therefore really worrying because many hospitals have only got small numbers of ICU beds and will rapidly run out. These patients may need artificial ventilation for several weeks!
Causes of Guillain-Barre syndrome
The exact causes of Guillain-Barre syndrome are still not known. The condition often develops a few days or weeks after a digestive tract or respiratory infection, however, suggesting that they may be related. Rarely, Guillain-Barre can arrive following surgery or immunization.
Some scientists believe that, somehow, the virus that precedes the onset of Guillain-Barre syndrome changes the cells of the nerves in some way, making them unrecognizable to the immune system and lining them up for attack.
Although a great deal of mystery still surrounds Guillain-Barre syndrome, there are some known risk factors:
The Zika virus is currently being suspected of causing Guillain-Barre syndrome.
- Males are slightly more likely to contract Guillain-Barre syndrome
- Risk increases with age
- Campylobacter infection (often from uncooked meat) sometimes precedes Guillain-Barre syndrome
- Influenza or Epstein-Barr virus
- HIV (human immunodeficiency virus)
- Mycoplasma pneumonia - a bacterial infection of the lungs
- Hodgkin's lymphoma - an uncommon cancer of the lymphatics
- Influenza or, rarely, childhood vaccinations.
Zika virus link
Recent findings reported in The Lancet suggest that the Zika virus might also cause Guillain-Barre syndrome.3
Zika virus has been previously associated with Guillain Barre syndrome and was first described in 2013 - 2014 when the incidence of Guillain Barre syndrome leapt by a factor of 8-fold during a concurrent Zika outbreak in the French Polynesian islands. This was the first evidence of such a link but experts commenting on the findings have referred to them as "compelling."4
We now find similar increases in the incidence of Guillain Barre syndrome in Latin America, where the reports of a recent Zika outbreak herald from. Zika has been associated with a mild flu-like illness in most that contract it but there have been reports of damage to unborn babies if contracted in utero and increasing incidence of microcephaly.
In 1976, there was a small increase in Guillain-Barre syndrome following a swine flu vaccination. However, the increase was just one additional case per 100,000. Additionally, research has shown that individuals are more at risk of developing Guillain-Barre syndrome after a case of the flu than they are from the vaccination itself.
According to the Centers for Disease Control and Prevention (CDC):5
"It is important to keep in mind that severe illness and death are associated with influenza, and vaccination is the best way to prevent influenza infection and its complications."
Diagnosis of Guillain-Barre syndrome
Guillain-Barre syndrome can be difficult to diagnose, especially in its earliest phases. The symptoms vary from person to person and are similar to those of other neurological conditions.
Initially, the doctor might ask whether the weakness is on both sides of the body - a feature of Guillain-Barre syndrome. The speed of onset is another clue; the progression of many neurological conditions is much slower than Guillain-Barre syndrome.
After a physical exam, the following tests may be recommended:
- Nerve conduction exam: electrodes are taped to the skin and the nerves are tested by passing small shocks through the skin. In Guillain-Barre syndrome, the signals travel along the nerves more slowly
- Electromyography: thin needle-like electrodes are used to test the functioning of muscle fibers
- Spinal tap (lumbar puncture): a sample of cerebrospinal fluid (CSF) is removed from the spinal canal and tested in a laboratory for specific signs of the disease. More protein is present in the CSF of people with Guillain-Barre syndrome.
Treatment of Guillain-Barre syndrome
Immunoglobulin therapy is not a cure but can help speed up recovery.
Although there are no known cures for Guillain-Barre syndrome, there are two types of treatment the can reduce the severity of its symptoms and improve the rate of recovery:
- Immunoglobulin therapy: immunoglobulins (also known as antibodies) from donors are given intravenously. This treatment seems to block the harmful antibodies involved in the autoimmune response
- Plasma exchange (plasmapheresis): blood is taken from the body, the plasma is removed and separated from the blood cells. The blood cells are then returned and the plasma is regenerated by the body. This process removes some of the antibodies that are attacking healthy cells.
Both methods are roughly as effective as each other, but using them together does not improve outcomes. Researchers do not yet know why either method works.
Prognosis for Guillain-Barre syndrome
In general, the disease worsens quickly for a couple of weeks and plateaus at around 4 months. The average recovery time for Guillain-Barre syndrome is 6-12 months.
Recovery from Guillain-Barre syndrome can be very slow, needing a lot of support, physiotherapy, counseling and occupational therapy. A few patients, sadly, are left with long term disability. Around 80% of patients can walk within 6 months, 60% fully recover strength within 1 year and 5-10% have incomplete or delayed recovery.
If you are already struggling with your New Year's resolution to eat healthier, a study published in the journal Autoimmunity Reviews may help you get back on the ladder; researchers suggest that eating processed foods may weaken the intestines in a way that raises the risk for autoimmune diseases, such as type 1 diabetes, celiac disease and multiple sclerosis.
Around 3 million people worldwide suffer from narcolepsy or bouts of sleepiness and sleep attacks that can affect their ability to have a normal life. There is no cure for the disorder, and few clues about its causes. But now, a new study suggests it could be an autoimmune disease.