Guillain-Barre syndrome occurs when the body’s immune system attacks part of its peripheral nervous system, causing symptoms that may lead to paralysis. Guillain-Barre syndrome is estimated to affect about 6 to 40 people out of each one million.
As noted, there are different types of Guillain-Barre in different parts of the world.
The type known as AIDP is most common in Europe and North America, resulting in about 90 percent of Guillain-Barre cases in those regions. In Asia and Latin America, AMAN and AMSAN account for 30 to 50 percent of cases (they are only three percent in the United States and Europe). And Miller Fisher syndrome, another variation of Guillain-Barre, is more common in Asian countries, pulling in 20 percent of cases in that area but accounting for less than five percent of them in Europe and North America.
MINOR BEGINNINGS
The disorder can start out somewhat mildly, with tingling or weakness in the legs. Eventually, those symptoms spread to the arms and upper body, increasing until muscles can’t be used, potentially cutting off the muscles that allow breathing and heart function.
There is no cure for the debilitating condition, and treatment usually involves being placed on a ventilator to assist with breathing.
Fortunately, the symptoms of Guillain-Barre syndrome can pass, and even severe cases have made good recoveries, albeit some with continued weakness in certain areas.
Guillain-Barre doesn’t discriminate. It can affect anyone at any age and of either sex, but the good news is that it’s so rare. It typically strikes after a patient has gastrointestinal or respiratory infection symptoms -- with the onset occurring anywhere from a few days to a few weeks after those diseases have seemingly passed. There is some evidence that certain vaccinations can increase the risk of having a Guillain-Barre attack.
When Guillain-Barre does strike, most people will be at the stage of greatest weakest within two weeks after the first symptoms appear, continuing into the third week of the symptoms’ appearance.
CAUSES OF GUILLAIN-BARRE
To date, there is no evidence that pinpoints exactly what causes Guillain-Barre syndrome. It is not contagious, but there is no clear indication of what triggers it and why it strikes some people and not others.
What researchers do know is that Guillain-Barre is an autoimmune disease, which causes the body’s immune system to begin attacking healthy people instead of invading bacteria or viruses. But in Guillain-Barre, the myelin sheath that surrounds the axons of peripheral nerves, and sometimes the axons themselves, are destroyed, cutting off nerve signals that cause body parts to fire. This injury or degradation means the signals that govern important body functions are cut off and can’t respond to brain commands. As the brain receives fewer signals, it loses its ability to feel heat, pain, textures and other sensations.
In the alternative, a sensation of crawling items, tingling or pain may be felt, usually hitting the extremities because they are further from the brain.
Researchers speculate that a virus or bacterial infection is the cause of Guillain-Barre syndrome. The infection may somehow mutate cells in the nervous system, attacking them when they turn “foreign” to the immune system sensors. It may also cloud those sensors so that it no longer discriminates on just what it will tolerate in the body.
To date, there is only speculation, and immunologists, virologists and neurological scientists are eagerly looking at what causes this mysterious affliction.
DIAGNOSIS AND TREATMENT
Guillain-Barre is called a syndrome because it is not yet clear that a specific disease is causing its appearance in humans. Research defines a syndrome as a medical condition characterized by a collection of symptoms (the patient’s role) and signs (the doctor’s observations and test results).
Because Guillain-Barre starts out with little by way of symptoms before progressing, it often gets rolling long before doctors recognize it. Several disorders mimic the symptoms of Guillain-Barre, delaying the diagnosis and testing. One of the most effective ways to diagnose Guillain-Barre is a spinal tap, but that procedure is done in operating rooms and usually must be scheduled. The onset of Guillain-Barre in its later stages, speeding up in days, often leaves little time to be certain.
Because there is no cure for Guillain-Barre syndrome, doctors must rely on ventilators and several therapies to slow down the severity of the syndrome and aid in recovery.
Among the therapies doctors use are plasma exchanges (referred to as plasmapheresis), a method in which whole blood is removed and processed so that the red and white cells are separated from the plasma and then returned to the patient. This helps the body quickly replace the plasma. The procedure appears to reduce the duration of the Guillain-Barre episodes and often heads off the most severe problems, Despite the effectiveness of the treatments, doctors have no idea why it works. Speculation is that the plasmapheresis removes antibodies and other immune system warriors that may be damaging the nerves.
Another successful therapy for Guillain-Barre is high-dose immunoglobulin therapy, which gives intravenous injections of the proteins the immune system uses to attack invading organisms. The proteins normally help the immune system battling invaders, and by taking donor-provided doses of allegedly healthy immunoglobulins, it seems to calm the immune system’s attack. Again, researchers are not sure why or how it works, but it does aid the patient.
Steroid hormones are also thought to help with immune system issues and lessen the severity. However, results in clinical trials have not been promising, and some believe the steroids may actually contribute to making the Guillain-Barre symptoms worse.
The key to treating this syndrome is to keep the patient alive and the body functioning in its breathing, heart rate and blood flow during the worst of the attack, giving the nervous system time to recover and fight back. This means hooking the patient up to an elaborate network of ventilator, heart monitor and other machines that help monitor or stimulate certain body parts. Most Guillain-Barre patients are placed in intensive care units, where they receive round-the-clock observation and treatment. Doctors can also watch for such side effects as pneumonia and bedsores that can accompany any paralysis.
While Guillain-Barre can offer some harrowing moments where the patient’s life may hang in the balance, those that survive it have a generally decent prognosis. Most recover fully, with about 30 percent exhibiting some weakness up to three years later. Some three percent of patients experience some relapse of Guillain-Barre, and about three to five percent can die, mostly because their chest muscles are paralyzed prior to hospitalization.