Treatment algorithm

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

ambulatory within 2 weeks of onset or non-ambulatory within 4 weeks of onset

1st line – intravenous immunoglobulin (IVIG)

Back
ACUTE
|
ambulatory within 2 weeks of onset or non-ambulatory within 4 weeks of onset
|
without IgA deficiency or renal failure
1st line – 

intravenous immunoglobulin (IVIG)

Plasma exchange and IVIG are equally efficacious. The choice between them is often institution-dependent. Combination therapy (plasma exchange followed by IVIG) is not recommended.[52][158][11]

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. 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.[158]

A randomised controlled trial found no evidence of any benefit of a second IVIG course for patients with GBS with a poor prognosis, and there was a risk of serious adverse events. Therefore a second course of IVIG is not recommended.[165]

Primary options

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

Plus – supportive treatment

Back
ACUTE
|
ambulatory within 2 weeks of onset or non-ambulatory within 4 weeks of onset
|
without IgA deficiency or renal failure
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.

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.[151]

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.[128][129] 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.[128][129][11] 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. 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.[125] 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.[151]

Tracheal intubation animated demonstration
Tracheal intubation animated demonstration

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

Bag-valve-mask ventilation animated demonstration
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: various medications (e.g., gabapentin, carbamazepine, amitriptyline) may be helpful in the acute and long-term management of neuropathic pain associated with GBS.[168] Opioids may aggravate autonomic gut dysmotility and bladder distension, and should be used with caution.[156][168][157]

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.[168][11] A multi-disciplinary approach has been shown to improve disability and quality of life, as well as reduce fatigue.[167]

1st line – plasma exchange

Back
ACUTE
|
ambulatory within 2 weeks of onset or non-ambulatory within 4 weeks of onset
|
without IgA deficiency or renal failure
1st line – 

plasma exchange

Plasma exchange and intravenous immunoglobulin (IVIG) are equally efficacious. The choice between them is often institution-dependent. Combination therapy (plasma exchange followed by IVIG) is not recommended.[52][158][11]

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.[50][166]

Two to five plasma exchanges are often needed, depending on the severity of GBS.[50]

The dose for plasma exchange, given through a central venous catheter, is 50 mL/kg bodyweight every other day for 7 to 14 days.[169]

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

Complications include severe infection, blood pressure instability, cardiac arrhythmias, and pulmonary embolus.[170][171] Compared with IVIG, plasma exchange showed more instances of pneumonia, atelectasis, thrombosis, and haemodynamic difficulties.[169] Other adverse effects include hypocalcaemia.

Plus – supportive treatment

Back
ACUTE
|
ambulatory within 2 weeks of onset or non-ambulatory within 4 weeks of onset
|
without IgA deficiency or renal failure
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.

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.[151]

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.[128][129] 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.[128][129][11] 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. 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.[125] 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.[151]

Tracheal intubation animated demonstration
Tracheal intubation animated demonstration

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

Bag-valve-mask ventilation animated demonstration
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: various medications (e.g., gabapentin, carbamazepine, amitriptyline) may be helpful in the acute and long-term management of neuropathic pain associated with GBS.[151] Opioids may aggravate autonomic gut dysmotility and bladder distension, and should be used with caution.[156][168][157]

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.[151][11] A multi-disciplinary approach has been shown to improve disability and quality of life, as well as reduce fatigue.[167]

1st line – plasma exchange

Back
ACUTE
|
ambulatory within 2 weeks of onset or non-ambulatory within 4 weeks of onset
|
with IgA deficiency or renal failure
1st line – 

plasma exchange

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

Ambulatory patients: plasma exchange is recommended within 2 weeks from the onset of neurological symptoms.

Non-ambulatory patients: plasma exchange is recommended within 4 weeks from onset.[158]

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.[50][166]

Two to five plasma exchanges are often needed, depending on the severity of GBS.[50]

The dose for plasma exchange, given through a central venous catheter (Mahurkar), is 50 mL/kg bodyweight every other day for 7 to 14 days.[169] During administration, patients should be closely monitored for electrolyte abnormalities and coagulopathies.

Complications include severe infection, blood pressure instability, cardiac arrhythmias, and pulmonary embolus.[170][171] Compared with IVIG, plasma exchange showed more instances of pneumonia, atelectasis, thrombosis, and haemodynamic difficulties.[169] Other adverse effects include hypocalcaemia.

Plus – supportive treatment

Back
ACUTE
|
ambulatory within 2 weeks of onset or non-ambulatory within 4 weeks of onset
|
with IgA deficiency or renal failure
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.

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.[151]

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.[128][129] 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.[128][129][11] 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. 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.[125] 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.[151]

Tracheal intubation animated demonstration
Tracheal intubation animated demonstration

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

Bag-valve-mask ventilation animated demonstration
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: various medications (e.g., gabapentin, carbamazepine, amitriptyline) may be helpful in the acute and long-term management of neuropathic pain associated with GBS.[151] Opioids may aggravate autonomic gut dysmotility and bladder distension, and should be used with caution.[156][168][157]

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.

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.[151][11] A multi-disciplinary approach has been shown to improve disability and quality of life, as well as reduce fatigue.[167]

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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

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