Guillain-Barre Syndrome describes a heterogeneous group of conditions characterised by distal, symmetrical paraesthesia followed by progressive limb weakness in both arms and legs and areflexia in the absence of any identifiable genetic, metabolic or toxic cause.
Other features which support a diagnosis of Guillain-Barre syndrome include:
- Progression of symptoms over days, up to 4 weeks
- Mild sensory symptoms or signs
- Cranial nerve involvement, especially bilateral weakness of facial muscles
- Autonomic dysfunction – hypo or hypertension, gastrointestinal dysmotility, arrhythmias
- Absent fever at onset
It is often preceded by infection:
- Campylobacter jejuni (around 25%)
- CMV
- Mycoplasma pneumonia
- EBV
- HIV
- Varicella zoster
Investigations:
- CSF – elevated protein with normal cell count (note CSF is initially normal in 50%) Electrodiagnostic testing – features of demyelination (slow conduction velocities), sural sparing pattern
The 5 subtypes of GBS are:
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
- Autoimmune disorder
- The ‘classic’ GBS and commonest form in western countries
- Acute motor axonal neuropathy (AMAN)
- Pure motor form
- High proportion of paediatric patients
- Commonest form in Japan and China
- Acute motor sensory axonal neuropathy (AMSAN)
- Minial inflammation and demyelination
- Miller Fisher syndrome
- Rare
- Rapidly evolving ataxia, areflexia, mild limb weakness, and ophthalmoplegia
- Acute panautonomic neuropathy
- Rarest of all
- Blurry vision and dry eyes
- Often cardiovascular involvement
Treatment
- Regular lung function tests to check for need for ventilatory support
- Ventilatory support
- Plasma exchange
- IVIG
- No evidence to support use of steroids
Prognosis
- 80-90% become non-ambulatory during the disease
- 50% have prominent pain
- 30% require ventilatory support
- Mortality 5%
- Full recovery in 85%
- Relapse in 5%
Factors suggesting poor outcome:
- Rapid onset
- Age >60
- Axonal degeneration
- C. jejuni infection
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