Introduction

Guillain-Barre syndrome describes an immune mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni)

Epidemiology

  • Incidence: 2.00 cases per 100,000 person-years
  • Peak incidence: 20-30 years
  • Sex ratio: more common in males 1.5:1
Condition Relative
incidence
Guillain-Barre syndrome1
Chronic inflammatory demyelinating polyneuropathy0.80
Transverse myelitis0.25
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Pathophysiology

Pathogenesis
  • cross reaction of antibodies with gangliosides in the peripheral nervous system
  • correlation between anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated
  • anti-GM1 antibodies in 25% of patients

Clinical features

The characteristic features of Guillain-Barre syndrome is progressive weakness of all four limbs. The weakness is classically ascending i.e. the lower extremities are affected first, however it tends to affect proximal muscles earlier than the distal ones. Sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs. Some patients experience back pain in the initial stages of the illness

Other features
  • there may be a history of gastroenteritis
  • areflexia
  • cranial nerve involvement e.g. diplopia
  • autonomic involvement: e.g. urinary retention, diarrhoea

Less common findings
  • papilloedema: thought to be secondary to reduced CSF resorption

Investigations

Investigations
  • lumbar puncture
    • rise in protein with a normal white blood cell count (albuminocytologic dissociation) - found in 66%
  • nerve condution studies may be performed

Differential diagnosis

Miller Fisher syndrome
  • variant of Guillain-Barre syndrome
  • associated with ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first
  • usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome
  • anti-GQ1b antibodies are present in 90% of cases

Management

Management
  • plasma exchange
  • IV immunoglobulins (IVIG): as effective as plasma exchange. No benefit in combining both treatments. IVIG may be easier to administer and tends to have fewer side-effects
  • steroids and immunosuppressants have not been shown to be beneficial
  • FVC regularly to monitor respiratory function

Prognosis

Poor prognostic features
  • age > 40 years
  • poor upper extremity muscle strength
  • previous history of a diarrhoeal illness (specifically Campylobacter jejuni)
  • high anti-GM1 antibody titre
  • need for ventilatory support

There is currently contradictory evidence as to whether a gradual or rapid onset of GBS is associated with a poor outcome

Prognosis
  • severe motor problems persist in about 15%
  • around 5% die