Guillain-Barre syndrome is a rare condition in which your body’s immune system attacks your nerves. The first symptoms are usually weakness and tingling in the extremities. These sensations can quickly expand and end up paralyzing the whole body. In its most severe form, Guillain-Barré syndrome is a medical emergency. Most sufferers require hospitalization for treatment.
The exact cause of Guillain-Barré syndrome is unknown. But two-thirds of patients report symptoms of an infection within six weeks. It can be a respiratory or gastrointestinal infection or the Zika virus. There is no known cure for Guillain-Barré syndrome, but several treatments can ease symptoms and shorten the duration of the illness. Although most people recover from Guillain-Barré syndrome, the mortality rate is 4-7%. Between 60 and 80% of people are able to walk after six months. Patients may experience lingering effects of the disease, such as weakness, numbness, or fatigue.
Symptoms of Guillain-Barre Syndrome
Guillain-Barré syndrome often begins with tingling and weakness that first manifests in the feet and legs, then spreads to the upper body and arms. In about 10% of people with the disease, symptoms begin in the arms or face. As Guillain-Barré syndrome progresses, muscle weakness can progress to paralysis.
Signs and symptoms of Guillain-Barré syndrome may include the following:
– Sensations of tingling, pins and needles in the fingers, toes, ankles or wrists.
– weakness in the legs that spreads to the upper part of the body
– Unsteady gait or inability to walk or climb stairs.
– Difficulty performing facial movements, including speaking, chewing or swallowing.
– Double vision or inability to move the eyes
– Severe pain which may be aching, shooting or cramp-like and may be worse at night.
– Difficulty controlling the bladder or bowels.
– Rapid heart rate
– Low or high blood pressure
– Difficulty breathing
People with Guillain-Barré syndrome usually feel their greatest weakness within two weeks of the onset of symptoms.
The 3 types of Guillain-Barre syndrome
While it was once thought to be a single disease, today Guillain-Barré syndrome is known to occur in many forms. The main types are:
1 Acute inflammatory demyelinating polyradiculoneuropathy (PIDA), the most common form in North America and Europe. The most common sign of AIDP is muscle weakness that starts in the lower part of your body and spreads upward.
2 Miller Fisher syndrome (MFS), in which paralysis begins in the eyes. FMS is also associated with an unsteady gait. FMS is less common in the United States and Europe but more common in Asia.
3 Acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN) are less common in the West but AMAN and AMSAN are more common in China, Japan and Mexico.
When to consult a doctor
Call your doctor if you experience mild tingling in your toes or fingers that doesn’t seem to spread or get worse. Seek emergency medical help if you have any of these serious signs or symptoms:
– Tingling that started in the feet or toes and moves up the body.
– Tingling or weakness that spreads quickly.
– Difficulty catching your breath or shortness of breath when lying down
– Choking by saliva
Guillain-Barré syndrome is a serious illness that requires immediate hospitalization because it can get worse quickly. The sooner appropriate treatment is instituted, the greater the chances of a good outcome.
Known causes of Guillain-Barre syndrome
The exact cause of Guillain-Barré syndrome is not known. The disorder usually appears a few days or weeks after an infection in the respiratory tract or digestive tract. In rare cases, recent surgery or vaccination can trigger Guillain-Barré syndrome. Recently, cases have been reported after Zika virus infection. Guillain-Barré syndrome can also occur after infection with COVID-19.
In the case of Guillain-Barré syndrome, the immune system, which usually only attacks invading organisms, begins to attack the nerves. In the case of AIDP, the most common form of Guillain-Barré syndrome, the protective sheath of the nerves (myelin sheath) is damaged. These lesions prevent nerves from transmitting signals to the brain, resulting in weakness, numbness or paralysis.
Guillain-Barre Syndrome Risk Factors
Guillain-Barré syndrome can affect all age groups. But the risk increases with age. It is also more common in men than in women.
Guillain-Barré syndrome can be triggered by:
– Most commonly infection with campylobacter, a type of bacteria often found in undercooked poultry.
– the flu virus
– cytomegalovirus
– the Epstein-Barr virus
– the Zika virus
– Hepatitis A, B, C and E
– HIV, the virus that causes AIDS
– Mycoplasma pneumonia
– Surgery
– Trauma
– Hodgkin’s lymphoma
– Rarely, flu vaccinations or childhood vaccinations
– COVID-19
Complications of Guillain-Barre Syndrome
Guillain-Barré syndrome affects your nerves. Because nerves control your movements and body functions, people with Guillain-Barré syndrome may experience:
breathing difficulties
The weakness or paralysis can spread to the muscles that control your breathing, a potentially fatal complication. Up to 22% of people with Guillain-Barré syndrome require temporary help from a breathing machine within the first week of being hospitalized for treatment.
Residual numbness or other sensations
Most people with Guillain-Barré syndrome recover completely or experience only minor residual weakness, numbness, or tingling.
Heart and blood pressure problems
Fluctuations in blood pressure and irregular heart rhythms (cardiac arrhythmias) are common side effects of Guillain-Barré syndrome.
Pain
A third of people with Guillain-Barré syndrome experience severe nerve pain, which can be relieved with medication.
Bowel and bladder function problems
Guillain-Barré syndrome can lead to slow bowel movement and urinary retention.
Blood clots
People who are immobile because of Guillain-Barré syndrome are at risk of developing blood clots. Until you are able to walk independently, taking blood thinners and wearing compression stockings may be recommended.
Bedsores
Immobility also puts you at risk of developing bedsores (pressure sores). Frequent repositioning can help avoid this problem.
Relapse
Two to five percent of people with Guillain-Barré syndrome experience a relapse.
Severe and early symptoms of Guillain-Barré syndrome greatly increase the risk of serious long-term complications. In rare cases, death can occur from complications such as respiratory distress syndrome and heart attacks.
Diagnostic
Guillain-Barré syndrome can be difficult to diagnose in its early stages. Its signs and symptoms are similar to those of other neurological disorders and may vary from person to person. Your doctor will likely start with a medical history review and a complete physical exam.
Your doctor may then recommend:
A lumbar puncture (lumbar puncture)
A small amount of fluid is taken from the spinal canal located in the lower back. This fluid is tested for a type of change that occurs frequently in people with Guillain-Barré syndrome.
Electromyography
Fine needle electrodes are inserted into the muscles your doctor wants to study. The electrodes measure nerve activity in the muscles.
Nerve conduction study. Electrodes are stuck on the skin above your nerves. A small shock is sent into the nerve to measure the speed of nerve signals.
Treatment of Guillain-Barre Syndrome
There is no treatment for Guillain-Barré syndrome. But two types of treatments can speed healing and reduce the severity of the disease:
Plasma exchange (plasmapheresis)
The liquid part of part of your blood (plasma) is removed and separated from your blood cells. The blood cells are then put back into your body, which makes more plasma to make up for what was removed. Plasmapheresis may work by clearing the plasma of certain antibodies that contribute to the immune system attacking peripheral nerves.
Immunoglobulin treatment
Immunoglobulin containing healthy antibodies from blood donors is given through a vein (intravenously). High doses of immunoglobulin can block harmful antibodies that can contribute to Guillain-Barré syndrome. These treatments are all equally effective. Mixing or administering them one after the other is no more effective than using either method alone.
Recovery and Healing
Although some people may take months or even years to recover, most people with Guillain-Barré syndrome follow this general timeline:
After the first signs and symptoms, the disease tends to get progressively worse for about two weeks.
Symptoms plateau in four weeks
Healing begins, and usually lasts six to twelve months, although for some people it can take up to three years.
Among adults who recover from Guillain-Barré syndrome:
About 80% can walk independently six months after diagnosis.
About 60% fully recover their motor strength one year after diagnosis.
About 5-10% have a very delayed and incomplete recovery.
Children, who rarely develop Guillain-Barré syndrome, usually recover more fully than adults.
Sources
Willison HJ, et al. Guillain-Barre syndrome. The Lancet. 2016;388:717.
Guillain-Barre syndrome fact sheet. National Institute of Neurological Disorders and Stroke.
McIntosh K. Coronavirus disease 2019 (COVID-19).