Guillain-Barre syndrome

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

non-ambulatory or severe bulbar weakness or autonomic instability within 4 weeks of onset; or ambulatory with mild disease within 2 weeks of onset

without IgA deficiency or renal failure

1st line – intravenous immunoglobulin (IVIG)

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1st line –

intravenous immunoglobulin (IVIG)

Treatment with IVIG should be initiated as soon as possible for non-ambulatory patients (GBS disability scale [GBS-DS] grade ≥3) within 4 weeks of symptom onset.[15] In patients who have relatively mild disease and are still able to walk unaided (GBS-DS grade 2), treatment with IVIG is recommended only in the first 2 weeks of symptom onset. Patients who are able to walk independently but have severe bulbar weakness or autonomic instability are eligible for IVIG within the first 4 weeks of symptom onset.[15]

Plasma exchange and IVIG are equally efficacious.[21][173][174] The choice between them is often institution-dependent. Combination therapy (plasma exchange followed by IVIG) is not recommended.[21][65][174]

IVIG is a pooled blood product and is associated with the risk of pathogen transmission (e.g., HIV, hepatitis B or C, Creutzfeldt-Jakob disease), although low. IVIG can precipitate anaphylaxis in an IgA-deficient person, and is contraindicated in these patients. However, it is much easier to administer than plasma exchange because it is a peripheral intravenous infusion. Treatment-related complications occur less frequently with IVIG than with plasma exchange.[174]

A minority of the patients treated with plasma exchange or IVIG for Guillain-Barre syndrome (GBS) can show worsening of symptoms after initial improvement or stabilisation.[171] This should raise the suspicion of a treatment-related fluctuation, in which case a repeated course of IVIG should be considered.[15] A second course of IVIG is not recommended for patients with GBS with a poor prognosis, as there is no evidence of benefit and an increased risk of serious adverse events.[172][173]

If patients show signs of worsening beyond 4 weeks, the possibility of acute-onset chronic inflammatory demyelinating polyradiculoneuropathy or another alternative diagnosis should be considered.[15]

Primary options

normal immunoglobulin human: 400 mg/kg/day intravenously for 5 days

Plus – supportive treatment

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Plus –

supportive treatment

Treatment recommended for ALL patients in selected patient group

All patients with severe disease should have their pulse and blood pressure (BP) monitored until they are off ventilator support and have begun to recover.[21][165]

Deep vein thrombosis prophylaxis: appropriate prophylactic anticoagulation (e.g., a direct oral anticoagulant, subcutaneous unfractionated heparin, or a low molecular weight heparin) and support stockings are recommended for non-ambulatory patients until they are able to walk independently.[165] See Venous thromboembolism (VTE) prophylaxis.

Respiratory management: risk factors for progression to mechanical ventilation include short time from symptom onset to hospital admission, bulbar, neck, or facial weakness, severe muscle weakness at hospital admission, and autonomic instability.[131][164] Algorithms or tools that predict a patient's risk of respiratory failure at admission (e.g., the Erasmus GBS Respiratory Insufficiency Score [EGRIS]) may be more reliable than individual variables.[21][131][132][133] Pulse oximetry and arterial blood gases should not be relied on, as hypoxia or hypercapnia is a late sign and patients will decompensate very quickly. Respiratory function should be monitored in all patients to quantify the risk of requiring mechanical ventilation and avoid emergency intubations.[15] Bedside spirometry including forced vital capacity should be monitored every 4-6 hours depending upon the degree of respiratory insufficiency. Single breath count is a useful bedside test; single breath count <20 predicts the need for ventilatory support.[15] Early intubation should be performed for patients with bulbar dysfunction, high risk of aspiration, and new atelectasis on chest x-ray. Elective intubation should be considered for patients with no or mild bulbar dysfunction if any of the following is present: vital capacity is <20 mL/kg; maximal inspiratory pressure is worse than -30 cmH₂O; maximal expiratory pressure is <40 cmH₂O; or vital capacity, maximal inspiratory pressure, or maximal expiratory pressure is reduced by 30% or more from baseline.[128] Once the patient is intubated, the need for tracheostomy should be addressed from week 2 onwards. If there is no improvement of pulmonary function tests (PFTs), percutaneous tracheostomy should be performed. If there is improvement of PFT above baseline, tracheostomy may be delayed for an additional week before reassessment.[165]

Tracheal intubation animated demonstration

How to insert a tracheal tube in an adult using a laryngoscope.

Bag-valve-mask ventilation animated demonstration

How to use bag-valve-mask apparatus to deliver ventilatory support to adults. Video demonstrates the two-person technique.

Pain: assess whether the cause of pain is neuropathic or nociceptive as treatment may differ.[15] Various drugs (e.g., gabapentin, carbamazepine, amitriptyline) may be helpful in the acute and long-term management of neuropathic pain associated with Guillain-Barre syndrome.[165] Guidelines recommend using carbamazepine as a second-line option after trying gabapentinoids (e.g., gabapentin) or tricyclic antidepressants (e.g., amitriptyline).[15]

Serotonin-noradrenaline reuptake inhibitors (SNRIs; e.g., duloxetine, venlafaxine) may also be used for long-term management of chronic neuropathic pain.[15] Opioids may aggravate autonomic gut dysmotility and bladder distension, and should be used with caution.[165][169][170]

Hypotension: can be managed with fluid boluses. Intra-arterial BP monitoring should be started if BP is very labile.

Hypertension: should be treated with short-acting agents (e.g., labetalol, esmolol, or nitroprusside) to prevent abrupt hypotension.

Rehabilitation: all patients should undergo an individual programme of rehabilitation in the acute phase, comprising gentle strengthening involving isometric, isotonic, isokinetic, and manual resistive and progressive resistive exercises. The focus is on proper limb positioning, posture, orthotics, and nutrition.[21][165] A multi-disciplinary approach has been shown to improve disability and quality of life, as well as reduce fatigue.[183]

1st line – plasma exchange

Back

1st line –

plasma exchange

Treatment with plasma exchange should be initiated as soon as possible for non-ambulatory patients (GBS disability scale [GBS-DS] grade ≥3) within 4 weeks of symptom onset.[15] In patients who have relatively mild disease and are still able to walk unaided (GBS-DS grade 2), treatment with plasma exchange is recommended only in the first 2 weeks of symptom onset. Patients who are able to walk independently but have severe bulbar weakness or autonomic instability are eligible for plasma exchange within the first 4 weeks of symptom onset.[15]

Plasma exchange and intravenous immunoglobulin (IVIG) are equally efficacious.[21][173][174] The choice between them is often institution-dependent. Combination therapy (plasma exchange followed by IVIG) is not recommended.[21][65][174]

Plasma exchange should be performed as early as possible. It is most effective if started within 7 days of symptom onset, but improvement in outcome has been observed when initiated up to 30 days after onset.[63][181]

Two to five plasma exchanges are often needed, depending on the severity of Guillain-Barre syndrome (GBS).[15][63]

A minority of the patients treated with plasma exchange for GBS can show worsening of symptoms after initial improvement or stabilisation.[171] This should raise the suspicion of a treatment-related fluctuation, in which case a repeated course of plasma exchange should be considered.[15] If patients show signs of worsening beyond 4 weeks, the possibility of acute-onset chronic inflammatory demyelinating polyradiculoneuropathy or another alternative diagnosis should be considered.[15]

The dose for plasma exchange, given through a central venous catheter, is 50 mL/kg bodyweight every other day for 7-14 days.[182]

During administration, patients should be closely monitored for electrolyte abnormalities and coagulopathies.

Complications include severe infection, blood pressure instability, cardiac arrhythmias, and pulmonary embolus.[63] Compared with IVIG, plasma exchange showed more instances of pneumonia, atelectasis, thrombosis, and haemodynamic difficulties.[182] Other adverse effects include hypocalcaemia.

Plus – supportive treatment

Back

Plus –

supportive treatment

Treatment recommended for ALL patients in selected patient group

All patients with severe disease should have their pulse and blood pressure (BP) monitored until they are off ventilator support and have begun to recover.[21][165]

Respiratory management: risk factors for progression to mechanical ventilation include short time from symptom onset to hospital admission, bulbar, neck, or facial weakness, severe muscle weakness at hospital admission, and autonomic instability.[131][164] Algorithms or tools that predict a patient's risk of respiratory failure at admission (e.g., the Erasmus GBS Respiratory Insufficiency Score [EGRIS]) may be more reliable than individual variables.[21][131][132][133] Pulse oximetry and arterial blood gases should not be relied on, as hypoxia or hypercapnia is a late sign and patients will decompensate very quickly. Respiratory function should be monitored in all patients to quantify the risk of requiring mechanical ventilation and avoid emergency intubations.[15] Bedside spirometry including forced vital capacity should be monitored every 4-6 hours depending upon the degree of respiratory insufficiency. Single breath count is a useful bedside test; single breath count <20 predicts the need for ventilatory support.[15] Early intubation should be performed for patients with bulbar dysfunction, high risk of aspiration, and new atelectasis on chest x-ray. Elective intubation should be considered for patients with no or mild bulbar dysfunction if any of the following is present: vital capacity is <20 mL/kg; maximal inspiratory pressure is worse than -30 cmH₂O; maximal expiratory pressure is <40 cmH₂O; or vital capacity, maximal inspiratory pressure, or maximal expiratory pressure is reduced by 30% or more from baseline.[128] Once the patient is intubated, the need for tracheostomy should be addressed from week 2 onwards. If there is no improvement of pulmonary function test (PFT), percutaneous tracheostomy should be performed. If there is improvement of PFT above baseline, tracheostomy may be delayed for an additional week before reassessment.[165]

Tracheal intubation animated demonstration

How to insert a tracheal tube in an adult using a laryngoscope.

Bag-valve-mask ventilation animated demonstration

How to use bag-valve-mask apparatus to deliver ventilatory support to adults. Video demonstrates the two-person technique.

Hypotension: can be managed with fluid boluses. Intra-arterial BP monitoring should be started if BP is very labile.

Hypertension: should be treated with short-acting agents (e.g., labetalol, esmolol, or nitroprusside) to prevent abrupt hypotension.

with IgA deficiency or renal failure

1st line – plasma exchange

Back

1st line –

plasma exchange

If there is a contraindication to intravenous immunoglobulin (IVIG) - namely, IgA deficiency or ongoing renal failure - plasma exchange is preferred over IVIG.

Treatment should be initiated as soon as possible for non-ambulatory patients (GBS disability scale [GBS-DS] grade ≥3) within 4 weeks of symptom onset.[15] In patients who have relatively mild disease and are still able to walk unaided (GBS-DS grade 2), treatment is recommended only in the first 2 weeks of symptom onset. Patients who are able to walk independently but have severe bulbar weakness or autonomic instability are eligible for plasma exchange within the first 4 weeks of symptom onset.[15]

Two to five plasma exchanges are often needed, depending on the severity of Guillain-Barre syndrome (GBS).[15][63]

The dose for plasma exchange, given through a central venous catheter (Mahurkar), is 50 mL/kg bodyweight every other day for 7-14 days.[182] During administration, patients should be closely monitored for electrolyte abnormalities and coagulopathies.

Plus – supportive treatment

Back

Plus –

supportive treatment

Treatment recommended for ALL patients in selected patient group

All patients with severe disease should have their pulse and blood pressure (BP) monitored until they are off ventilator support and have begun to recover.[21][165]

Respiratory management: risk factors for progression to mechanical ventilation include short time from symptom onset to hospital admission, bulbar, neck, or facial weakness, severe muscle weakness at hospital admission, and autonomic instability.[131][164] Algorithms or tools that predict a patient's risk of respiratory failure at admission (e.g., the Erasmus GBS Respiratory Insufficiency Score [EGRIS]) may be more reliable than individual variables.[21][131][132][133] Pulse oximetry and arterial blood gases should not be relied on, as hypoxia or hypercapnia is a late sign and patients will decompensate very quickly. Respiratory function should be monitored in all patients to quantify the risk of requiring mechanical ventilation and avoid emergency intubations.[15] Bedside spirometry including forced vital capacity should be monitored every 4-6 hours depending upon the degree of respiratory insufficiency. Single breath count is a useful bedside test; single breath count <20 predicts the need for ventilatory support.[15] Early intubation should be performed for patients with bulbar dysfunction, high risk of aspiration, and new atelectasis on chest x-ray. Elective intubation should be considered for patients with no or mild bulbar dysfunction if any of the following is present: vital capacity is <20 mL/kg; maximal inspiratory pressure is worse than -30 cmH₂O; maximal expiratory pressure is <40 cmH₂O; or vital capacity, maximal inspiratory pressure, or maximal expiratory pressure is reduced by 30% or more from baseline.[128] Once the patient is intubated, the need for tracheostomy should be addressed from week 2 onwards. If there is no improvement of pulmonary function test (PFT), percutaneous tracheostomy should be performed. If there is improvement of PFT above baseline, tracheostomy may be delayed for an additional week before reassessment.[165]

Tracheal intubation animated demonstration

How to insert a tracheal tube in an adult using a laryngoscope.

Bag-valve-mask ventilation animated demonstration

How to use bag-valve-mask apparatus to deliver ventilatory support to adults. Video demonstrates the two-person technique.

Hypotension: this can be managed with fluid boluses. Intra-arterial BP monitoring should be started if BP is very labile.

Hypertension: should be treated with short-acting agents (e.g., labetalol, esmolol, or nitroprusside) to prevent abrupt hypotension.

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Guillain-Barre syndrome

Treatment algorithm

non-ambulatory or severe bulbar weakness or autonomic instability within 4 weeks of onset; or ambulatory with mild disease within 2 weeks of onset

intravenous immunoglobulin (IVIG)

Primary options

supportive treatment

plasma exchange

supportive treatment

plasma exchange

supportive treatment