According to a Swedish epidemiologic study, 4 the incidence appears to be lower during pregnancy with an increase in the months immediately after delivery. The patient with GBS typically presents with weakness accompanied by tingling dysesthesias in the extremities.
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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.
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. Pain is most severe in the shoulder girdle, back, and posterior thighs. Pain may be accompanied by muscle cramps, and it is most severe at night.
GBS is the most common type of rapidly evolving, generalized peripheral nervous system disorder. However, several disorders can be mistaken for GBS, particularly early in its course Table 1. The diagnosis of GBS is based on typical clinical features; electrodiagnostic examination and examination of the cerebrospinal fluid CSF can aid in the diagnosis Table 2.
These findings may allow earlier intervention with specific treatments, but a definitive diagnosis is usually not possible until the fifth day after the onset of symptoms. Hypophosphatemia irritable, apprehensive, hyperventilation, normal cerebrospinal fluid.
Information from Ropper AH. N Engl J Med ;—6. Characteristic CSF findings consist of elevated protein higher than 0.
CSF is often normal when symptoms have been present for less than 48 hours, but by the end of one week the level of CSF protein is elevated. High concentration of protein in cerebrospinal fluid, with fewer than 10 cells per cubic millimeter.
Reprinted with permission from Ropper AH. Ann Neurol ;27 suppl :S21—4. The axonal forms are generally thought to have poorer prognoses, indicating a need for determining the specific subtype. These subtypes are distinguished electrodiagnostically and pathologically Table 3. Acute inflammatory demyelinating polyradiculoneuropathy AIDP. Electrophysiologic studies are normal in sensory nerves, reduced or absent in motor nerves. Reduced or absent sensory nerve action potentials, tibial H reflex is usually absent.
Cardiovascular involvement is common postural hypotension, tachycardia, hypertension, dysrhythmias. Information from references 2 , and 12 through Acute infectious illnesses are well-known antecedent events in two thirds of patients who have GBS.
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Patients commonly report a respiratory tract infection or gastroenteritis that resolved when the neuropathy began. Campylobacteriosis is the most common precipitant in GBS. In , results of a case-control study of patients with GBS found that 26 percent of affected persons were positive for C. Seventy percent of the patients with C. Recent influenza immunization also has been associated with GBS.
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One study reviewed the cases of GBS during the — and — influenza seasons and found an adjusted relative risk of 1. Food and Drug Administration to monitor reports of suspected adverse events after immunization, concluded in its surveillance summary that the risk of developing vaccine-associated GBS is less than the risk of severe influenza. In addition, despite increasing doses of administered vaccine during the four previous influenza seasons, the number of GBS reports has remained stable.
Treatment of GBS has two components: supportive care and specific therapy. Supportive care remains the cornerstone of therapy. If patients advance past the acute phase of illness, most will recover function.
However, the neuropathy can advance so rapidly that endotracheal intubation and mechanical ventilation may be necessary within 24 hours of symptom onset. For this reason, all patients who have GBS should be admitted to a hospital for close observation for respiratory compromise, cranial nerve dysfunction, and autonomic instability.
Autonomic nervous system dysfunction may manifest as fluctuations in blood pressure, cardiac dysrhythmias, gastrointestinal pseudo-obstruction, and urinary retention. Progression is noted with reduction of more than 30 percent in vital capacity, maximal inspiratory pressure, or maximal expiratory pressure. Arch Neurol ;—8. As respiratory muscles weaken, elective endotracheal intubation should be considered.
Progression to respiratory failure can be predicted using measurable respiratory parameters Table 4. Frequent reassessment with serial lung function testing for rapid progression is critical. Additional predictors of subsequent mechanical ventilation include the following: 1 time from GBS onset to hospital admission of less than seven days, 2 inability to lift the elbows or head above the bed, 3 inability to stand, 4 ineffective coughing, and 5 increased liver enzyme levels. Predictors of mechanical ventilation in patients who had a previously determined vital capacity included time from GBS onset to admission of less than seven days, an inability to lift the head, and a vital capacity less than 60 percent predicted.
One retrospective study 21 demonstrated a 40 percent decrease from predicted vital capacity, compared with a 60 percent decrease reported in another study. Pain and psychologic stress should be treated. Narcotics should be used with caution because risk of ileus is already increased. Physical therapy, including gentle massage, passive range-of-motion exercises, and frequent position changes may provide pain relief. Carbamazepine 23 Tegretol and gabapentin 24 Neurontin have been used as adjuncts in pain management in GBS.
Patients who were treated with these medications required less narcotic analgesia with fewer narcotic side effects and minimal sedation compared with those who received placebo. Patients are paralyzed by the illness, but mentally alert and fearful. Reassurance and discussion about the phases of illness and recovery can help reduce psychologic stress. Specific treatment should be initiated soon after diagnosis. High-dose intravenous immunoglobulin IVIg; mg per kg daily for five days or plasmapheresis five exchanges over five to eight days can be initiated. To determine whether IVIg was as effective as plasma exchange in treating patients with GBS, a large multicenter trial 25 was designed to compare plasma exchange and IVIg and the combination of both treatments for GBS.
The study followed patients over four weeks. There were no statistically significant differences in the disability rating between the two treatment groups. IVIg and plasmapheresis were found to be equally effective therapies.
A Cochrane Database Review combined the largest trials into a meta-analysis and also concluded that IVIg is as effective as plasma exchange in hastening recovery from GBS in patients who required assistance to walk. In another multicenter RCT of approximately patients, 28 there was no additional benefit from the combination of therapies, and the rate of relapse was not statistically significant. Patients treated early with plasmapheresis required less mechanical ventilation, and hospitalization time was decreased. The French Cooperative Group on Plasma Exchange in GBS concluded that patients with mild symptoms of GBS at admission benefit from two plasma exchanges while patients with more severe symptoms may require two additional plasma exchanges.
There is some disagreement regarding the optimal number of plasma exchanges, but all of the studies showing a benefit used four to six exchanges. Plasma exchange is the only treatment that is superior to supportive treatment alone. Disadvantages of plasmapheresis include its rare complications, such as sepsis, that are believed to be caused by depletion of immunoglobulins. IVIg treatment has advantages over plasmapheresis because it is easier to administer, has significantly fewer complications, and is more comfortable for the patient. IVIg is recommended for patients with GBS who cannot ambulate without assistance within two to four weeks of neuropathic symptom onset.
Pathologic antibodies might be bound by the IgG increasing their clearance. Despite its benefits, there are side effects from this IVIg therapy. IVIg expands the plasma volume so it must be administered with caution in patients with congestive heart failure and renal insufficiency. A history of previous anaphylaxis to IVIg is a contraindication to repeat treatment.
The risk of serious hepatitis C infection transmission has been reduced because of changes in preparation and purification. Corticosteroids were once believed to be useful in the treatment of GBS because of its immune-mediated inflammatory mechanism. However, a Cochrane Database Review of randomized trials, which included patients treated with corticosteroids compared with controls, showed no difference in outcome.
Approximately 85 percent of patients with GBS achieve a full and functional recovery within six to 12 months. Recovery is maximal by 18 months past onset. However, some patients have persistent minor weakness, areflexia, and paresthesia. Approximately 7 to 15 percent of patients have permanent neurologic sequelae including bilateral footdrop, intrinsic hand muscle wasting, sensory ataxia, and dysesthesia. Several factors during the acute phase of illness predict subsequent poor recovery. These factors include age older than 60 years; severe, rapidly progressive disease; and low nerve conduction amplitudes on distal stimulation, which suggests axonal loss.
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In general, a poor long-term prognosis is directly related to the severity of the acute episode and delay in onset of specific treatment. Relapse occurs in a small percentage of patients. One multicenter trial 18 of patients showed a relapse rate of 3 to 5 percent. In that study, the relapse rate was not significantly affected by treatment type or any other factor tested. Already a member or subscriber? Log in. Newswanger received her osteopathic degree from the Philadelphia College of Osteopathic Medicine, Philadelphia, and completed a residency in family medicine at Naval Hospital Jacksonville, Jacksonville, Fla.
Address correspondence to Dana L.
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Reprints are not available from the authors. The authors indicate that they do not have any conflicts of interest.