Introduction
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 syndrome | 1 |
Chronic inflammatory demyelinating polyneuropathy | 0.80 |
Transverse myelitis | 0.25 |
<1 | 1-5 | 6+ | 16+ | 30+ | 40+ | 50+ | 60+ | 70+ | 80+ |
Pathophysiology
- 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
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
- 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
- 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
- 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
- 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