Guillain-Barré syndromeBMJ 2008; 337 doi: https://doi.org/10.1136/bmj.a671 (Published 17 July 2008) Cite this as: BMJ 2008;337:a671
- John B Winer, consultant neurologist
- 1University Hospital Birmingham, Queen Elizabeth Hospital, Edgbaston, Birmingham B15 2TH
Guillain-Barré syndrome is a rare but important disease that can lead to life threatening respiratory failure
Structural similarities between a triggering infectious organism and peripheral nerve tissue are important in its pathogenesis
Treatment consists of rapid administration of intravenous immunoglobulin or plasma exchange, which shortens the time to recovery
Around 10% of patients die from respiratory failure, pulmonary emboli, or infection
Around 20% of patients have residual disability, with weakness or persistent sensory disturbance
Guillain-Barré syndrome is a peripheral neuropathy that causes acute neuromuscular failure. Misdiagnosis is common and can be fatal because of the high frequency of respiratory failure, which contributes to the 10% mortality seen in prospective studies.1 Our understanding of the wide spectrum of the disease and its pathogenesis has increased enormously in recent years. Several high quality randomised controlled trials have established the effectiveness of early treatment.
Sources and selection criteria
I prepared this review by searching Cochrane reviews, Medline, PubMed, and my personal archive of references. I downloaded and assessed all references that dealt with Guillain-Barré syndrome and its subtypes—acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, and acute motor and sensory axonal neuropathy.
What is the spectrum of Guillain-Barré syndrome?
The clinical spectrum of Guillain-Barré syndrome is varied—at least three different types have been identified. In Europe and North America about 95% of cases are acute inflammatory demyelinating polyradiculoneuropathy and the other 5% are acute axonal motor disorder and acute sensory and motor axonal neuropathy.2 The frequency of these axonal neuropathies varies throughout the world, and in Asia and South America they make up about 30% of the syndrome.3 The closely related Miller Fisher syndrome is thought to be an inflammatory neuropathy that affects the cranial nerves to the eye muscles in particular, and it is characterised by ophthalmoplegia, accompanied by areflexia and ataxia but not weakness.4 Some cases of acute …