Guillain-Barré Syndrome - American Family Physician

with weakness accompanied by tingling dysesthesias in the extremities. This weakness is prominent in the proximal muscles; legs are more often affected than arms. Paresthesias occur, spreading proximally but seldom extending past the wrists and ankles. Deep tendon reflexes disappear within the first few days of symptom onset. The progressive phase of the syndrome lasts from a few days to four weeks. About 73 percent of patients reach a nadir of clinical function at one week and 98 percent at four weeks. The progressive phase is followed by a plateau phase of persistent, unchanging symptoms. Improvement will begin within days of the plateau. The time to resolution of symptoms varies among patients. Cranial nerve involvement may affect airway maintenance, facial muscles, eye movements, and swallowing. Patients should be hospitalized for observation. Approximately 30 percent of patients will require ventilatory assistance at some time during the illness. Poor outcomes primarily are associated with the increasing severity of disease, with a mortality rate as high as 20 percent occurring primarily in patients who require mechanical ventilation. Pain, another common feature of GBS, is seen in approximately one half of all patients and is sometimes described as severe, occurG uillain-Barré syndrome (GBS) is an eponym for a heterogeneous group of immune-mediated peripheral neuropathies. A feature common in all GBS variants is a rapidly evolving polyradiculoneuropathy preceded by a triggering event, most often an infection. GBS generally manifests as a symmetric motor paralysis with or without sensory and autonomic disturbances. Population-based surveys attempting to document the annual incidence of GBS have been conducted in various countries worldwide and generally are in agreement on a rate of 1 to 3 per 100,000 persons annually. GBS occurs in all age groups, although rarely in infants, and the incidence varies. From birth to 30 years, the annual incidence is fairly uniform at 1.3 to 1.9 per 100,000. Peaks are noted in late adolescence and young adulthood, as well as in the elderly. The first peak likely correlates with increased risk of cytomegalovirus and Campylobacter jejuni infection. The reason for the peak in the elderly is unknown but is postulated to be caused by failing immune suppressor mechanisms. Another variation in incidence is found in pregnant and postpartum women. According to a Swedish epidemiologic study, the incidence appears to be lower during pregnancy with an increase in the months immediately after delivery. Guillain-Barré syndrome (GBS) is a group of autoimmune syndromes consisting of demyelinating and acute axonal degenerating forms of the disease. Nerve conduction study helps differentiate the heterogeneous subtypes of GBS. Patients exhibit a progressive paralysis that reaches a plateau phase. In most patients, resolution is complete or near complete. Mortality from GBS most often is associated with dysautonomia and mechanical ventilation. GBS usually is associated with an antecedent infection by one of several known pathogens. Cross-reactivity between the pathogen and the nerve tissue sets up the autoimmune response. Treatment consists of supportive care, ventilatory management (in about one third of patients), and specific therapy with intravenous immunoglobulin or plasmapheresis. Consultation with a neurologist is suggested. (Am Fam Physician 2004;69:2405-10. Copyright© 2004 American Academy of Family Physicians) Guillain-Barré Syndrome