By Peter Galvin, MD
Guillain-Barre syndrome (GBS) is a rare neurological disease that affects the peripheral nerves and causes muscle weakness. It is an autoimmune disease that affects the nerves outside of the brain and spinal cord (the peripheral nerves) and develops over several days to weeks. GBS can cause severe muscle weakness, and death occurs in about 5% of cases. The most common subtypes are acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Approximately 90% of people with GBS in North America and Europe have AIDP. Patients with the AIDP subtype of GBS typically have weakness that starts in the legs and spreads to the arms, as well as decreased or absent reflexes. In more than 50% of these patients, nerves that originate in the brain stem (cranial nerves) are affected, which may cause facial weakness, difficulty swallowing, and eye muscle weakness or paralysis. About 25% to 30% of patients develop severe weakness or paralysis of the muscles used to breathe. GBS typically causes symptoms of low back pain and limb numbness and tingling, and fluctuations in blood pressure or an irregular heart rhythm can also occur.
GBS affects people worldwide, and the lifetime risk of GBS is estimated at one in 1000. Although it may occur at any age, the incidence increases with age, and males are slightly more likely to develop it than females. Approximately two thirds of patients have a diarrheal or respiratory illness four to six weeks prior to the onset of GBS symptoms. Other, less common events or conditions that may trigger GBS include recent surgery, pregnancy, or immunosuppression. Although rare, sporadic cases have been reported after vaccinations, but the risk of developing postvaccination GBS is much lower than the risk of developing GBS after an infection. The diagnosis of GBS is made based on symptoms and physical examination findings. Neurological testing may include electromyography and nerve conduction tests to assess nerve and muscle function. The results of a lumbar puncture (spinal tap) may support the diagnosis of GBS and can rule out other neurological diseases.
Those with suspected GBS should be admitted to the hospital as close monitoring of their breathing, heart rate, and blood pressure is required. Individuals who develop severe respiratory muscle weakness or paralysis are supported with mechanical ventilation. Those with swallowing issues can receive nutrition through a feeding tube. Current treatment recommendations include intravenous immune globulin, an infusion of antibodies, or plasma exchange (plasmapheresis) which involves removal and replacement of the liquid component of blood. About 40% to 50% of patients with GBS do not improve within four weeks and require prolonged supportive care. Physical, speech, and occupational therapy are important to help patients regain strength and function.
Most patients with GBS gradually improve and have complete recovery over six to 12 months. Some, unfortunately, have prolonged residual symptoms, including pain, fatigue, numbness, tingling, and muscle weakness. Some factors associated with a higher risk of death due to GBS include older age, more severe disease, and prolonged need for mechanical ventilation.
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