Guillain-Barré syndrome (GBS) is the leading cause of flaccid paralysis in western countries. It is a self-limiting, autoimmune, neurologic disease that presents as rapidly progressive symmetric limb weakness, loss of tendon reflexes and autonomic dysfunction. While the cause of GBS is still unclear, various infections have been established as triggers. The most frequent antecedent pathogen is Campylobacter jejuni, but Cytomegalovirus and various vaccinations have also been implicated in the etiology of GBS. A review by Hahn discusses the epidemiology, progression and treatment of the disease.
Patients require supportive therapy that is appropriate to the extent of their symptoms. Approximately one third of patients with GBS require intensive care and ventilatory support. Autonomic dysfunction and hemodynamic instability are common, requiring vigilance for the potential complications of thromboembolism, cardiac dysfunction, ventilatory failure and oropharyngeal weakness. Patients may need total supportive care, including pain and symptom control, skin and bowel care, hydration and nutritional support, and psychologic services. Intravenous steroid therapy has not been shown to be beneficial in the treatment of GBS.
Plasma exchange can be effective if used within two weeks of symptom onset. At least three controlled trials have reported a more rapid return to ambulation, fewer days on ventilation support and in the hospital, and lower costs for patients treated with plasma exchange. After an initial improvement, approximately 10 percent of patients experience secondary relapse within two weeks of starting plasma exchange. Current recommendations range from two plasma exchange treatments for mild GBS to five treatments for more severe cases. Plasma exchange is expensive and has significant risks, particularly in hemodynamically unstable patients.
Intravenous IgG therapy, usually given in a dosage of 0.4 g per kg for five days, has a lower risk and is easier to administer than plasma exchange. Two studies have concluded that IgG therapy yields results comparable to those of plasma exchange and that combining the two therapies does not confer a significant advantage. Relapses also occur in approximately 10 percent of patients treated with IgG therapy.
The authors conclude that although the management of GBS can be challenging and may require intensive therapy, the prognosis is usually favorable. The progression of the disease is highly variable. Approximately 75 percent of cases reach maximal dysfunction within two weeks, and 94 percent reach it within four weeks. Symptoms resolve gradually. In one series, 70 percent of patients reported full recovery within one year; 22 percent of patients were unable to run, and 8 percent could not walk without assistance. The percentage of patients who die from GBS varies among studies but is approximately 7 percent. Patients who are older than 60 years are at greatest risk of death and serious long-term complications. A preceding diarrheal illness and rapid disease progression are also associated with poor prognosis.