For the sections on pathogenesis and diagnosis, we searched MEDLINE and EMBASE in all languages on Nov 4, 2004, and our personal databases, using the search term “Guillain-Barré syndome”. For the sections on treatment, we searched the Cochrane Library and made use of the relevant Cochrane reviews which themselves used the published search strategy and methods for selecting evidence of the Cochrane Neuromuscular Disease Group.
SeminarGuillain-Barré syndrome
Section snippets
Worldwide incidence
The incidence of typical Guillain-Barré syndrome has been reported to be relatively uniform between 0·6 and four cases per 100 000 per year throughout the world,16 but the most recent and careful population-based studies in Europe consistently report an incidence of 1·2–1·9 per 100 000.17, 18, 19, 20, 21, 22 Atypical cases such as Fisher's syndrome are much less common and Italian researchers have reported an incidence of 0·1 per 100 000.18 All reports agree that men are about 1·5 times more
Diagnosis
The diagnosis of Guillain-Barré syndrome itself is usually not difficult for the neurologist, but can be challenging for the doctor of first contact who may not have seen a case since medical school. Established diagnostic criteria exist and have stood the test of time.37 Most patients will have an acute neuropathy reaching a peak in under 4 weeks, weakness, hyporeflexia or areflexia, and raised protein concentrations in CSF. However, the rapid development of inexplicable weakness in a patient
Neurophysiological testing
Neurophysiological studies play a very important role in diagnosis, subtype classification, and confirmation that the disease is a peripheral neuropathy (panel 2).9, 45 Sufficient information is required: usually, this would include data from at least three sensory nerves, at least three motor nerves with multisite stimulation and F waves, and bilateral tibial H-reflexes. In some cases, information from a smaller number of nerves may suffice. With this neurophysiological information, individual
Investigations
In addition to neurophysiological testing, a lumbar puncture procedure is traditional and almost always appropriate. A raised CSF protein concentration is present in about 80% of patients, but CSF protein content is more likely to be normal during the first days of the illness.10, 14 CSF should be analysed before treatment with intravenous immunoglobulin (IVIg), which can cause aseptic meningitis. Other investigations may be needed to exclude causes of similar illnesses or to identify
AIDP
The classic pathological picture of Guillain-Barré syndrome is of multifocal mononuclear cell infiltration throughout the peripheral nervous system in which the distribution of inflammation corresponds to the clinical deficit.3 Macrophages invade the myelin sheaths and denude the axons. For the most part, macrophages seem to invade intact myelin sheaths (figure 1), as occurs in experimental autoimmune neuritis.4, 63, 64 According to one hypothesis, the activated macrophages are targeted to
Clinical course
In typical cases, the first symptoms are pain, numbness, paraesthesia, or weakness in the limbs. The weakness may initially be proximal, distal, or a combination of both. Numbness and paraesthesia usually affect the extremities and spread proximally. In children, pain may be a prominent presenting symptom. The facial nerves are often affected and less often the bulbar and ocular motor nerves. In 25% of cases, weakness of the respiratory muscles requires artificial ventilation. Autonomic
Prognosis
From the many case series, and especially from population-based studies that have investigated possible prognostic factors, the most consistent finding has been that the outlook is worse in elderly patients.9, 19, 140 In children, recovery is more rapid and more likely to be complete; death is exceptional.143, 144 In adults and children, severity of disease at nadir, expressed as being bedbound or requiring artificial ventilation, has usually been identified as an adverse prognostic factor.140
General treatment
Excellent multidisciplinary care is needed to prevent and manage the potentially fatal complications of the disease, and the methods have been the subject of a consensus report.147 Respiratory failure occurs in 25% of patients and is more likely in cases with rapid progression, bulbar palsy, upper limb involvement, and autonomic dysfunction. Regular monitoring, including measurement of vital capacity, and early transfer to an intensive therapy unit for prophylactic intubation are essential. All
Immunotherapy
Plasma exchange became accepted as the gold standard treatment for Guillain-Barré syndrome almost 20 years ago. Evidence to support this practice has accumulated from six trials, but not all studies provided all the outcome measures of interest. Most used a 7-point Guillain-Barré syndrome disability grade scale. In four trials, including 585 participants with available data, plasma exchange increased the improvement after 4 weeks by an average of 0·89 grades (95% CI 0·63–1·14). In five trials
Search strategy and selection criteria
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