What is Guillain Barré Syndrome?
This Neurologic Condition can be Associated with Vaccinations
Apr 29, 2009
Guillain-Barré syndrome is an acquired inflammatory nerve disorder; it is the most common acquired inflammatory neuropathy, affecting up to four in 100,000 persons annually. The condition causes respiratory failure in 25% of affected individuals, permanent disability in 20%, persistent fatigue in 67%, and death in 4 to 15%. (Clinical Briefs: Immunotherapy in Patients with Guillain-Barré Syndrome. Am Fam Phys 2004;69(4):997)
The cause of Guillain-Barré is poorly understood, but it apparently leads to autoimmune injury to nerve tissue or to the tissues that support the nerves. In about two-thirds of patients, the syndrome begins several days to weeks after an infectious disease, a surgical procedure, or a vaccination.
Infectious agents that have been associated with Guillain-Barré include Campylobacter jejuni, herpesviruses such as Epstein-Barr and cytomegalovirus, Mycoplasma, and several enteric viruses.
Reports of vaccine-associated Guillain-Barré occur sporadically; in 1976, a cluster of cases occurred during the swine flu vaccination program.
Signs and Symptoms of Guillain-Barré Syndrome
- Onset is characterized by symmetric weakness and abnormal sensations, usually beginning in the legs and progressing to the arms; occasionally, symptoms begin in the arms or head.
- Weakness worsens over several days to weeks, with flaccidity becoming more prominent than sensory abnormalities in most patients.
- Deep tendon reflexes disappear, but sphincter control is usually maintained.
- Facial and oropharyngeal muscles are weakened in more severe cases.
- Respiratory paralysis requiring mechanical ventilation occurs in up to 10% of cases.
- Weakness becomes maximal in 90% of affected persons at around three weeks.
- Uncommon variants of the syndrome may cause autonomic dysfunction (blood pressure fluctuation, cardiac dysrhythmias, urinary retention, cessation of gastrointestinal motility, pupillary changes, etc.) or isolated weakness of eye muscles with poor coordination and loss of reflexes.
How is Guillain-Barré Syndrome Diagnosed and Treated?
- Guillain-Barré must be distinguished from other causes of acute weakness, such as polio, tick paralysis, myasthenia gravis, metabolic conditions, or West Nile virus. Diagnosis is mainly based on clinical examination and history.
- If Guillain-Barré syndrome is suspected, patients must be admitted to a hospital, as this condition constitutes a medical emergency. Constant monitoring of respiratory function and muscular activity are necessary to anticipate the need for respiratory support.
- Physical therapy during the acute phase of the illness helps prevent contractures and other orthopedic problems. Severely affected patients require turning and padding to prevent decubitus ulcers.
- Plasmapharesis (replacement of the patient’s plasma with albumin or fresh plasma) slows progression of Guillain-Barré syndrome when performed early in the illness. This treatment also shortens the course and reduces the risks of death or permanent paralysis.
- Gamma globulin is also beneficial when administered early in the course of the illness; the effects of plasmapharesis and gamma globulin are equivalent, and combining the two therapies does not confer any added benefit. The choice of treatment often depends on the ready availability of one modality over the other.
Prognosis for Guillain-Barré Syndrome
- Most patients improve significantly over several months, and many recover full function. However, 30% of adults and an even larger number of children retain some degree of residual weakness when evaluated three years after the illness.
- Persons with residual deficits often need retraining, occupational therapy, orthopedic appliances, or surgery to correct contractures.
- After initial improvement, up to 10% of patients relapse and develop a chronic syndrome of slowly progressive weakness.
(From The Merck Manual, 18th Edition, 2006. Guillain-Barré syndrome. Pp 1894-96)
Guillain-Barré syndrome can be a cause of significant disability or even death. The risk of persons acquiring this condition must be weighed in the development of new vaccines, particularly when such vaccines are brought to the market quickly in order to avert epidemics or pandemics.
