Guillain-Barre syndrome

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

A multidisciplinary approach to the acute phase is required, combining supportive therapy and disease-modifying therapy with either high-dose intravenous immunoglobulin (IVIG) or plasma exchange.[98] IVIG and plasma exchange are equally efficacious. This topic focuses on the management of Guillain-Barre syndrome (GBS) in adults.

Supportive therapy: respiratory management

Respiratory failure is common in GBS, and approximately 20% to 30% of patients need ventilatory support in an intensive care unit (ICU).[98][164]

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. The single breath count test (assessing the patient’s ability to count whilst holding their breath) is a useful bedside test; single breath count value of <20 predicts the need for ventilatory support.[15]

Patients with bulbar dysfunction, high risk of aspiration (i.e., infiltrates on chest x-ray), and new atelectasis on chest x-ray should be intubated early for airway protection and impending respiratory failure.

In patients with no bulbar dysfunction, or with mild bulbar dysfunction without aspiration risk, the 20/30/40 rule should be used.[128] The patient should be monitored in the ICU and elective intubation considered if any of the following is present:

Vital capacity is <20 mL/kg
Maximal inspiratory pressure is worse than -30 cmH₂O (negative inspiratory force)
Maximal expiratory pressure is <40 cmH₂O
Vital capacity, maximal inspiratory pressure, or maximal expiratory pressure is reduced by 30% or more from baseline[128]

The reported mean duration of ventilation is 15-43 days, and weaning should be guided by serial pulmonary function tests (PFTs) and assessment of strength.[165] The need for tracheostomy should be addressed from week 2 onwards, especially if PFTs do not show improvement. If there is improvement of PFTs above baseline, tracheostomy may be delayed by 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.

Nasopharyngeal airway animated demonstration

How to select the correct size naspharyngeal airway and insert the airway device safely.

Oropharyngeal airway animated demonstration

How to size and insert an oropharygeal airway.

Supportive therapy: cardiovascular management

Haemodynamic monitoring of pulse and blood pressure (BP) should be started on admission. Telemetry is prudent, especially if there is evidence of dysautonomia. If dysautonomia is present, continuous cardiac monitoring and placement of a Foley catheter should be initiated on admission. There are insufficient data for methods and setting of monitors, but all patients with severe disease should have their pulse and BP monitored until they are off ventilator support and have begun to recover.[21][165]

Fluid balance should be monitored carefully, because the autonomic dysfunction renders clinical determination of hydration status very difficult. Hypotensive episodes can be managed with fluid boluses.

If BP is very labile, intra-arterial BP monitoring should be initiated. Hypertensive episodes should be treated with short-acting agents (e.g., labetalol, esmolol, nitroprusside) to prevent abrupt hypotension.

Other factors that may potentiate dysautonomia include manoeuvres such as suctioning and changing position (i.e., lying to sitting), and drugs (antihypertensive drugs, succinylcholine).[168]

Supportive therapy: deep vein thrombosis (DVT) prophylaxis

Immobility and hypercoagulability from treatments such as IVIG can increase the risk of DVT in these patients.[21][65] 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.

Supportive therapy: pain management

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 GBS.[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]

Indications for immunotherapy

Immunotherapy in GBS is guided by two main factors: the severity of disease and the duration since symptom onset.

In patients with symptom onset within 4 weeks who cannot walk unaided (GBS disability scale [GBS-DS] grade ≥3), treatment with either IVIG or plasma exchange should be initiated as soon as possible.[15] In patients who have relatively mild disease and are still able to walk unaided (GBS-DS grade 2), treatment with plasma exchange or IVIG is recommended only in the first 2 weeks of symptom onset. Patients who are able to walk unaided but have severe bulbar weakness or autonomic instability are eligible for plasma exchange or IVIG within the first 4 weeks of symptom onset.[15]

A minority of the patients treated with plasma exchange or IVIG 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 IVIG or plasma exchange 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]

Choice of immunotherapy

Immunotherapy comprises IVIG or plasma exchange.

IVIG and plasma exchange 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 administered via peripheral intravenous infusion. It is used more frequently than plasma exchange because plasma exchange requires central venous access and is associated with tolerability issues.[175] Treatment-related complications occur less frequently with IVIG than with plasma exchange.[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 the risk is low.

IVIG can precipitate anaphylaxis in an IgA-deficient person, and is contraindicated in these patients. Plasma exchange is preferred in the presence of ongoing renal failure.

There is no evidence concerning the relative efficacy of plasma exchange and IVIG for treating axonal forms of GBS.

Corticosteroids are not recommended for treatment of GBS.[15] Corticosteroid monotherapy does not significantly shorten time to recovery or prevent long-term disability in patients with GBS; oral corticosteroids delay recovery compared with placebo, possibly due to harmful effects on denervated muscle.[176] [ ] [Evidence B]

Intravenous immunoglobulin (IVIG)

One Cochrane review found moderate-quality evidence that, in severe disease, IVIG given within 2 weeks of disease onset expedites recovery to a similar extent as plasma exchange.[65] Although there are no randomised controlled trial (RCT) data on the efficacy of IVIG >2 weeks from the onset of symptoms, the European Academy of Neurology/Peripheral Nerve Society guideline suggests that IVIG can still be considered between 2-4 weeks based on the evidence that IVIG and plasma exchange are equally effective within 2 weeks.[15]

One small retrospective study did not find a difference in outcomes for two groups given IVIG either within 7 days of symptom onset or 7 days or more after symptom onset.[177]

Plasma exchange (plasmapheresis)

Large RCTs have established the effectiveness of plasma exchange in severe disease.[178][179][180] Evidence suggests that in adults with GBS, plasma exchange is superior to supportive care with respect to:[63]

Mean time to recover walking with aid (primary outcome)
Shorter time to onset of recovery (primary outcome)
Improvement by one disability grade by 4 weeks (secondary outcome)

Plasma exchange is most effective if started within 7 days of symptom onset, but improvement in outcome has been observed when plasma exchange was initiated up to 30 days after onset.[63][181]

Plasma exchange should be initiated in parallel with supportive care. Two to five plasma exchanges are often needed, depending on the severity of GBS.[15][63] Patients undergoing plasma exchange 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.

The risk of relapse during the first 6-12 months after symptom onset is higher with plasma exchange compared with those not treated.[63]

Rehabilitation

This is recommended in the acute phase. It comprises gentle strengthening involving isometric, isotonic, isokinetic, and manual resistive and progressive resistive exercises. Rehabilitation should be focused 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]

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