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Guillain-Barré Syndrome (GBS)

Created: 14/7/2008
Updated: 15/7/2008

Guillain-Barré Syndrome (GBS)

Advanced Trainee in Intensive Care Medicine & Anaesthetics
Oxford Radcliffe Hospitals NHS Trust

Focus on background & pathophysiology of GBS

Guillain-Barré syndrome (GBS) was characterised in 1916 as a constellation of symptoms of motor weakness, parasthesiae and muscle tenderness, with associated increased cerebrospinal spinal fluid (CSF) protein. Indeed, 90% of patients will have a CSF protein level greater than 0.4g/L after 1 week. Many variants of GBS have since been described, the most common being acute inflammatory demyelinating polyradiculopathy (AIDP). GBS can therefore be regarded as a heterogeneous group of immunologically mediated disorders of peripheral nerve function. The reported incidence is 1.7 per 100,000, with a slight preponderance for males and the elderly. The immunological basis of the nerve injury is cell-mediated pathways, with demyelination being the primary pathological process. Autoantibodies to nervous system components can be demonstrated in some patients. Prior sensitisation by an antecedent infection is thought to occur (Table 1). In patients presenting with GBS, 43% present within weeks of a respiratory illness, and 21% after gastrointestinal disturbance. Evidence of Campylobacter infection is apparent in 26-41% of GBS patients.

Table 1. Commonly implicated antecedent infective agents in GBS

 Influenza A       
 Campylobacter jejunii


Focus on the presentation of GBS

The prodrome typically occurs between 0 to 8 weeks, with a peak interval of around 2 weeks. The initial presentation is of parasthesiae in a glove and stocking distribution in around 50% of patients with GBS, while 25% will complain of motor weakness. The remainder of GBS patients will describe both motor and sensory symptoms, with motor weakness becoming the predominant issue. This motor weakness can progress to flaccid paralysis.

Clinical examination reveals a reduction in both power and deep tendon reflexes. Cranial nerve deficits are apparent in 45% of cases, with VII, IX and X being the most frequently affected. The Miller-Fisher variant is a well-described form of GBS cranial nerve abnormality, accompanied by ataxia, areflexia and ophthalmoplegia. Another variant is acute motor sensory axonal neuropathy (AMSAN), where the axons, rather than the myelin sheath, are the primary targets of damage. Both the Miller-Fisher and AMSAN variants are associated with preceding Campylobacter infection. Sensory loss is often mild and predominantly pertains to those modalities moderated via the dorsal columns.

Focus on the principles of ICU management of GBS

The ICU management of GBS broadly consists of:

• Specific therapeutic interventions
• General supportive measures in the interim of disease resolution
• The avoidance of complications and iatrogenic insult

Focus on specific therapeutic interventions for GBS

Specific treatment options for GBS centre on plasmapheresis (plasma exchange) and immunoglobulin therapy. Plasmapheresis has been shown to reduce morbidity, but not necessarily mortality, if commenced within 7 days of onset of symptoms. Immunoglobulin has been shown to be as effective as plasmapheresis and is usually given as 5 daily doses of 0.4g/kg. In approximately 10% of patients relapse occurs and a second treatment is required. Immunoglobulin therapy has increased in popularity over plasmapheresis, mainly due to its superior side-effect profile and the fact that it can be given in non-specialist centres.

Focus on general ICU supportive measures for GBS

Respiratory support
Patients who remain spontaneously ventilating require a programme of chest physiotherapy and close monitoring of respiratory function. Methods of assessment include repeated assessment of either forced vital capacity (FVC; by far the most common approach) and/or arterial blood gases. An FVC of less than 15ml/kg (or less than 30% of FVC predicted) or a rising PaCO2 are indications for mechanical ventilation. Other markers of respiratory compromise include maximal inspiratory pressure <30 cmH2O and maximal expiratory pressure <40 cmH2O. Around 30% of GBS patients will need ventilatory support, and this can often be prolonged. Many intensivists will consider undertaking a tracheostomy at an early stage. One recognised advantage of an ‘early’ tracheostomy is a significant reduction in the amount of sedation that is required. Post-intubation pulmonary function testing may inform the need for tracheostomy.

Autonomic dysfunction
Autonomic dysfunction is a major cause of morbidity and mortality, particularly in ventilated patients. This autonomic dysfunction may result in features such as refractory orthostatic hypotension, paroxysmal hypertension, bradycardia, ventricular tachyarrhythmias, ileus and urinary retention. Autonomic dysfunction is of particular importance at the induction of anaesthesia, which can be a significant challenge. Careful consideration should be given to the use of suxamethonium and inotropic and vasopressor agents may produce markedly atypical responses in heart rate and blood pressure. Even tracheal suction may lead to significant cardiovascular instability.

Gastrointestinal support should include nasogastric feeding wherever possible. Problems may occur when an ileus is present, and the need for airway protection should again be considered when bulbar palsy and gastric reflux is a possibility. Total parenteral nutrition may occasionally be necessary when enteral measures prove inadequate.

Musculoskeletal features may include limb/girdle pain, for which simple analgesics, quinine, anti-depressants and opioids have all been used successfully. Nerve conduction studies may show reduced velocities and prolonged distal latencies. Physiotherapy should continue throughout the illness to prevent the sequelae of limb disuse, with careful attention given to pressure area care. Deep-vein thrombosis prophylaxis is considered a priority.

Psychological issues
GBS patients frequently require a great deal of psychological support, given the extremely disabling and frightening aspects of the condition and its sequelae. Undertaking a multidisciplinary approach to GBS patients is an important aspect of overall ICU management, but is particularly useful in addressing patients’ psychological well-being.

Focus on the prognosis of GBS

Patients with GBS typically experience the worst of the symptoms between 2 and 4 weeks after onset. At 1 year, 70% of GBS patients have made a full recovery, but up to 20% retain some physical limitation. However, improvement can continue for up to 2 years. Overall mortality of GBS is 5-8%. A poorer prognosis is associated with being elderly, mechanically ventilated or in Campylobacter-associated cases.

Key Learning Points

• Patients with severe forms of GBS often require ICU admission and present a real challenge in terms of ICU management.
• Specific ICU treatments and interventions can result in significant additional patient morbidity.
• Respiratory morbidity predominates in patients with severe GBS admitted to the ICU.
• ICU follow-up of GBS patients is warranted in view of the considerable longer-term physical and psychological problems.

Key References

Henderson RD, Lawn ND et al. The morbidity of Guillain-Barré syndrome admitted to the intensive care unit. Neurology 2003; 60: 17-21.

Richards K. Guillain-Barré syndrome. Br J Anaesth 2003; 3: 246-9.

Pearse RM. Non-invasive ventilation to avoid tracheal intubation in a patient with Guillain-Barré syndrome. Br J Anaesth 2003; 91: 913-16.

van Der Meché FG, van Doorn PA. Guillain-Barré Syndrome. Curr Treat Options Neurol 2000; 2(6): 507-16.

Hughes RA, Swan AV, Raphaël JC, Annane D et al. Immunotherapy for Guillain-Barré syndrome: a systematic review. Brain 2007; 130: 2245-57.

Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Lancet 1997; 349(9047): 225-30.

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