Guillain-Barré syndrome (GBS) is a rare autoimmune disorder where the body’s immune system mistakenly damages its peripheral nerves, which are located outside the brain and spinal cord. GBS is not contagious or inherited and can affect people of all ages, though it is more common in adults and males. The condition often requires immediate medical attention as symptoms can progress quickly.
Causes and Triggers
The precise cause of Guillain-Barré syndrome is not fully understood, but its onset often follows a recent infection or other event by about five days to three weeks. In response to a trigger, the immune system mistakenly attacks the body’s own nerves. This autoimmune attack targets the myelin sheath, the protective covering of the nerves, which disrupts the transmission of nerve signals to the brain.
The most frequently identified trigger is an infection with the bacterium Campylobacter jejuni, a common cause of foodborne illness. Viral infections are also common precursors, including influenza, cytomegalovirus, Epstein-Barr virus, and the Zika virus. In some instances, the syndrome has been linked to recent surgery or, very rarely, vaccinations. Despite these associations, a specific trigger is not always identified.
Symptoms and Progression
Initial symptoms of Guillain-Barré syndrome include weakness and tingling, often described as pins and needles, that start in the feet and legs. The condition progresses in a pattern of ascending paralysis, where the weakness moves upward through the body over days or weeks. In 90% of cases, the weakness reaches its most severe point within three to four weeks after symptoms first appear.
As the syndrome advances, weakness can spread to the arms, torso, and facial muscles, affecting the ability to speak, chew, or swallow. In serious cases, GBS can impact the muscles that control breathing, and up to a third of individuals require mechanical ventilation. While the ascending pattern is most common, some people may first notice symptoms in their arms or face. A less common variant, Miller Fisher syndrome, can present with issues in eye movement, coordination, and balance first.
The Diagnostic Process
Diagnosing Guillain-Barré syndrome involves a careful neurological examination and specific tests to rule out other conditions with similar symptoms. Doctors assess for muscle weakness and absent or diminished deep tendon reflexes as indicators of the syndrome.
Two diagnostic tools are used to confirm GBS. One is a lumbar puncture, or spinal tap, where cerebrospinal fluid is collected from the lower back; in GBS, this fluid typically shows high protein with a normal white blood cell count. The other procedure is nerve conduction studies, which may include electromyography (EMG). These tests measure nerve signal speed and strength, which are often slowed in GBS due to myelin sheath damage.
Treatment Approaches
There is no cure for Guillain-Barré syndrome, but medical treatments can lessen the severity and shorten the duration of the illness. Treatments focus on managing the autoimmune response and are administered in a hospital. Patients require close monitoring of breathing, heart rate, and blood pressure.
The two main immunotherapies are plasma exchange (plasmapheresis) and intravenous immunoglobulin (IVIG) therapy. In plasma exchange, blood is removed from the body, and the liquid portion (plasma) containing the harmful antibodies is separated from the blood cells, which are then returned to the body. Intravenous immunoglobulin therapy involves administering high doses of healthy antibodies from blood donors, which helps block the damaging antibodies attacking the nerves. Supportive care, such as using blood thinners to prevent clots, is also part of treatment.
Recovery and Outlook
Recovery from Guillain-Barré syndrome can be a lengthy process, taking several months or even years. After the acute phase of the illness, which lasts up to eight weeks, patients may require extensive rehabilitation to regain muscle strength and movement. This involves a team of specialists providing physical, occupational, and sometimes speech therapy.
The prognosis for individuals with GBS varies. Most people recover fully or almost fully, though it may take six months or longer to walk again. However, some are left with long-term effects like persistent weakness, numbness, or fatigue, and about 30% of adults experience residual weakness after three years. While recovery is the norm, the condition can be life-threatening in severe stages, with a small percentage of patients dying from complications like respiratory failure or cardiac arrest.