BMJ, 17 July 2008
By: John B Winer, consultant neurologist, University Hospital Birmingham, UK.
What are the clinical features?
- All types of Guillain-Barré syndrome present with acute neuropathy, defined as: progressive onset of limb weakness that reaches its worst within four weeks.
- Typically there are sensory symptoms but few sensory signs.
- Reflexes are usually lost early in the illness.
- The respiratory system is affected in a third of cases, but this may not be associated with clear dyspnoea, which makes it more difficult to assess.
Summary points:
- Guillain-Barré syndrome is a rare but important disease that can lead to life threatening respiratory failure. (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).
- 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.
