Guillain-Barre Syndrome – RCEMLearning EMFP

Author: Richard Loffhagen / Editor: Jason M Kendall / Reviewer: Kaja Rasheed, John Wilson, Jia Luen Goh / Codes: A5 / Published: 27/12/2021 / Reviewed: 25/05/2026

Introduction

Context

Almost a century ago French neurologists Guillain and Barr gave their name to Guillain-Barr Syndrome (GBS), after they described the cases of two soldiers who presented with acute paralysis and areflexia, and later recovered. GBS is an acute immune mediated polyneuropathy with several variant forms.¹

Patients classically present with weakness and sensory symptoms that start in the legs and progresses to the arms rapidly, which can also affect the cranial nerves. 20-30% of patients may develop respiratory failure and may require ventilatory support.²

The incidence of the disease is static at 12/100,000. It is more common in men (1.5:1) and with advancing age.¹

In spite of advances in the understanding of the pathogenesis and treatment options, it remains a severe disease, which can be difficult to diagnose and treat.

Pathophysiology and Variant Forms

The pathophysiology of GBS is thought to be an autoimmune response against peripheral nerve components.¹

The most common variant bilaterally affects motor and sensory nerves.

The key findings in GBS are profoundly-delayed conduction in nerve fibres, secondary to axonal demyelination. This occurs primarily in peripheral nerves and spinal roots, but may affect cranial nerves as well.

There is growing evidence that, at least in some cases, GBS is caused by an aberrant immune response to an infective trigger.

The subtypes of GBS³:

Acute inflammatory demyelinating polyneuropathy (AIDP): targets myelin sheat, Schwann-cell components
Acute motor axonal neuropathy (AMAN): targets axonal membrane
Acute motor sensory axonal neuropathy (AMSAN)

Table 1 Variants of GBS⁴

Variant	Frequency (% of cases)	Clinical features
Classic sensorimotor GBS	30-85	Rapidly progressive symmetrical weakness and sensory signs with absent or reduced tendon reflexes, usually reaching nadir within 2 weeks
Pure motor	570	Motor weakness without sensory signs
Miller Fisher syndrome	5-25	Ophthalmoplegia, ataxia and areflexia. Incomplete forms with isolated ataxia (acute ataxic neuropathy) or ophthalmoplegia (acute ophthalmoplegia) can occur³¹. Overlaps with classical sensorimotor GBS in an estimated 15% of patients
Paraparetic	5-10	Paresis restricted to the legs
Pharyngealcervicalbrachial	<5	Weakness of pharyngeal, cervical and brachial muscles without lower limb weakness
Bilateral facial palsy with paraesthesias	<5	Bilateral facial weakness, paraesthesias and reduced reflexes
Bickerstaff brainstem encephalitis^d	<5	Ophthalmoplegia, ataxia, areflexia, pyramidal tract signs and impaired consciousness, often overlapping with sensorimotor GBS
Pure sensory	<1	Acute or subacute sensory neuropathy without other deficits

Fig. 1 Pattern of symptoms in variants of GBS⁴

Clinical Assessment

Clinical Features

The diagnostic features of GBS are summarised below:

GBS characteristics

GBS is characterised by a rapidly-progressive, bilateral weakness, accompanied by reduced or absent tendon reflexes.⁵ The symptoms and signs of GBS typically, but not invariably, move proximally.

Diagnostic features^1,5

Progressive weakness in both arms and legs, often starting with legs
Reduced, or absent, tendon reflexes
Relative symmetry of symptoms
Progression of symptoms up to four weeks
Most patients experience partial or complete recovery
Absence of fever at presentation
Mild sensory symptoms or signs
Facial weakness or other cranial nerve involvement
Pain

Precipitants

The most common presentation is a post-infectious disorder in an otherwise healthy patient. Approximately two-thirds of patients report symptoms of an infection (gastrointestinal or upper respiratory) in the preceding three weeks.¹

Organisms implicated in preceding infections include²:

Bacteria

Campylobacter jejuni (predominant cause, 30% patients)
Haemophilus influenzae
Mycoplasma pneumoniae (predominant in children)

Viruses

Cytomegalovirus
Epstein-Barr virus
Hepatitis
HIV
Zika Virus

Early symptoms

Early symptoms are pain, numbness, weakness or paraesthesia in the limbs, followed by a rapidly progressive symmetrical bilateral weakness. This may extend to respiratory muscles or those innervated by cranial nerves.⁵

Whilst the typical pattern of ascending weakness is well described, the weakness may affect arms and/or legs, and may take on a variety of patterns.¹

There are generally decreased or absent tendon reflexes in affected limbs.

Labratory findings

Albuminocytological dissociation on CSF analysis after a week of symptom onset is seen in about 66% of patients. This is characterised by raised Cerebrospinal fluid (CSF) protein (>0.4 g/L), with normal white cell count (WCC).⁵

Progression of GBS

The weakness will reach its maximum within four weeks of onset and, in most cases, does so within two weeks.

There is normally a plateau period of varying length followed by a slow recovery phase of weeks, months or even years.⁶

Disease progression beyond 8 weeks is classified as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Differential Diagnosis

In countries where poliomyelitis has been eradicated, GBS remains the most common cause of acquired paralysis in children. For a classical presentation, making the diagnosis is relatively simple. However, presentation may be subtle and includes minor changes of gait or refusal to walk, associated with pain.^1,5

Learning Bite

The emergency physician needs to keep GBS in the differential diagnosis of the limping child.⁵

Factors which make GBS a less likely diagnosis include a fever at presentation, raised cerebrospinal fluid white cell count (CSF WCC), an atypical presentation, or history of travel to developing countries.

GBS Mimics

A comprehensive list of differential diagnoses which may mimic the neurological presentation of GBS is shown below.^1,5,6

Intracranial/spinal cord abnormalities

Brainstem encephalitis
Meningitis
Carcinomatosis/lymphomatosis
Transverse myelitis
Cord compression

Anterior horn cell abnormalities

Poliomyelitis
West Nile virus

Spinal nerve root abnormalities

Compression
Inflammation (e.g. cytomegalovirus)
Leptomeningeal malignancy

Peripheral nerve abnormalities

There are many causes of peripheral neuropathy:

Chronic inflammatory demyelinating polyneuropathy (CIDP)
Drug-induced neuropathy
Porphyria
Critical illness polyneuropathy
Vasculitis
Diphtheria
Vitamin B1 deficiency (Beriberi)
Heavy metal or drug intoxication
Tick paralysis
Metabolic disturbances (hypokalaemia, hypophosphataemia, hypermagnesaemia, hypoglycaemia)

Neuromuscular junction abnormalities

Myasthenia gravis
Botulism
Organophosphate poisoning

Muscular abnormalities

Critical illness polyneuromyopathy
Polymyositis
Dermatomyositis
Acute rhabdomyolysis

Investigation Strategies

The diagnosis of GBS is largely clinical, but the standard investigations are listed below.

The key diagnostic tests for the ED are the lumbar puncture, along with a screen of blood tests.^1,6

Lung function tests are vital for safe management of the patient.⁵

Investigations

Acute investigations include^1,5:

Blood tests: FBC, ESR, U+E, LFT, CPK, CRP, glucose, Mg, calcium and phosphate
Stool culture (for C jejuni and others)
ECG
Lumbar puncture
Lung function tests if any respiratory compromise, or unable to walk

Other diagnostic tests include:

MRI may have a place in diagnosis. Selective anterior nerve root enhancement appears to be strongly suggestive of GBS. The cauda equina nerve roots are enhanced in 83% of patients
Nerve conduction and other electrodiagnostic studies may confirm and classify the diagnosis and is also used to prognosticate.
Immunological tests: Antibodies to GQ1B may confirm a Miller Fisher variant.
Nerve or muscle biopsy
Specific disease related tests e.g. HIV test

Interpretation of results

Learning Bite

A raised CSF protein (>0.4 g/L) with normal white cell count is the typical CSF finding in GBS.1,5

However, CSF protein may be normal in the first week of the disease, but will have increased in more than 90% of cases by the end of the second week.^5,6

A raised serum CK suggests an alternative diagnosis such as a myositis.

Hypokalaemia and hypoglycaemia may also mimic GBS symptoms, and should be excluded.

Liver function tests are often mildly elevated in GBS.

Serial lung function tests are important as the onset of respiratory compromise can be rapid. Patients with an FVC less than 20mL/kg are candidates for prophylactic intubation and mechanical ventilation.^5,6

There is much current work regarding immunological studies in GBS. These may confirm an immune pathogenesis, and may affect long-term treatment in some cases, but currently have little place in the acute diagnosis, or treatment, of GBS.

Management

Initial Treatment

Initial treatment is supportive. Plasma exchange (PE) or intravenous immunoglobulins (IVIG) are indicated in the treatment of GBS patients in the following patients:

Unable to walk independently for 10m
Rapidly deteriorating patients
Patients with dysphagia or respiratory failure

Corticosteroids, either as single treatment or in addition to IVIg, did not show any associations with improved outcome in RCTs.

Treatment Options

Monitoring

Lung function initially every four hours
ECG
BP
Autonomic function pupils and ileus
Check for swallowing dysfunction

General measures

Pain relief: try to avoid opioids
Chronic neuropathic pain: amitriptyline or antiepileptic drugs
DVT prophylaxis
Eye care
Meticulous nursing to avoid pressure sores
Physiotherapy to avoid contractures
Consider early ICU admission for severely affected patients

Specific treatments

IVIG or PE for those unable to walk, ideally to start within two weeks of onset of symptoms
Typical IVIG regimen: 0.4g/kg for five days
Typical PE regimen: total exchange of five plasma volumes in two weeks
Retreatment with IVIG may be indicated if there is a secondary deterioration

Indications for ICU admission

Risk of aspiration pneumonia
Requiring ventilated support
Rapidly-progressive disease
Labile heart rate or blood pressure

The Erasmus GBS Respiratory Insufficiency Score (EGRIS) is a validated risk prediction tool to predict the probability of respiratory insufficiency within the first week of admission. An EGRIS score of 57 suggests 65% (5476%) probability of respiratory insufficiency in the first week of admission.^7,8

A modified EGRIS (mEGRIS) is a simplified version that can be used up to a month after onset of symptoms (this has not been externally validated).

The 20/30/40 rule to anticipate the need for mechanical ventilation has also been used with some success^7,8:

vital capacity <20 ml kg¹,
maximal inspiratory pressure <30 cmH₂O
maximum expiratory pressure <40 cmH₂O

The management of GBS in summary is as follows⁸:

Complications and Prognosis

Early complications are common, particularly during the acute progressive stage.¹

Autonomic dysfunction occurs in up to two-thirds of patients, and can lead to cardiac arrhythmias and blood pressure fluctuations. This can occasionally be associated with sudden death. Patient may require a pacemaker to manage severe bradyarrhythmias.¹

Invasive ventilation may be required and this is the best predictor for extended hospital stay with some patients requiring tracheostomy. However, this does not predict outcome. Common inpatient complications include pneumonia and tracheobronchitis.

Other common complications

DVT and PE
Pressure ulcers
Pain
Psychological delirium, anxiety, depression, PTSD

Increasing age, history of preceding diarrhoea at time of presentation and high level of motor weakness at the diseases worst are poor prognostic factors for the ability to walk independently during first 6 months of follow up.

Approximately one quarter of severely-affected patients require ventilatory support.⁵

Even with IVIG or PE, one fifth of those severely affected remain unable to walk at six months.

Prognosis can be predicted from the acute stage of the disease, based on:

Increasing patient age
Severity of disability
History of preceding diarrhoea
These are all poor prognostic factors.

Learning bite

GBS is fatal in 3-10% of cases. The cause of death is multifactorial.⁶

Key Learning Points

GBS is a spectrum of diseases affecting both the axon and myelin sheath.
A post-infectious presentation is common, particularly Campylobacter Jejuni gastroenteritis.
Typical GBS presents with rapidly progressive symmetrical ascending weakness and reduced or absent reflexes.
Presentation may be subtle, especially in children, with pain, gait disturbance, or refusal to walk. GBS should be considered in the differential diagnosis of the limping child.
Fever at presentation, raised CSF white cell count (WCC), atypical features, or relevant travel history should prompt consideration of alternative diagnoses.
All suspected cases should be referred for admission
ICU care may be required for patients with significant respiratory problems
ECG and blood pressure monitoring are required
Severe cases should be treated with IVIG
Corticosteroids have no proven benefit in GBS.
Prognosis in children is generally very good

References

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Weiss N, et al. Critical insights for intensivists on Guillain-Barr syndrome. Ann Intensive Care. 2025 May 21;15(1):67.
Leonhard SE, et al. Diagnosis and management of Guillain-Barr syndrome in ten steps. Nat Rev Neurol. 2019 Nov;15(11):671-683.
BMJ Best Practice. Guillain-Barr syndrome. BMJ Publishing Group, 2026.
Bhatti KS, Maheshwary A, Sun CE. Guillain-Barre Syndrome. [Updated 2026 Jan 31]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2026 Jan-.
van Doorn PA, et al. European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barr syndrome. Eur J Neurol. 2023 Dec;30(12):3646-3674.
Willison HJ, et al. Guillain-Barr syndrome. Lancet. 2016 Aug 13;388(10045):717-27.
Guillain G, Barr JA, Strohl A. Sur un syndrome de radiculo-nvrite avec hyperalbuminose du liquide cphalo-rachidien sans raction cellulaire. Remarques sur les caractres cliniques et graphiques des rflexes tendineux [Radiculoneuritis syndrome with hyperalbuminosis of cerebrospinal fluid without cellular reaction. Notes on clinical features and graphs of tendon reflexes. 1916]. Ann Med Interne (Paris). 1999 Jan;150(1):24-32. French. PMID: 10400560.
Yang YR, et al. Comparative proteomics analysis of cerebrospinal fluid of patients with Guillain-Barr syndrome. Cell Mol Neurobiol. 2008 Aug;28(5):737-44.
Hughes RA, Swan AV, Raphal JC, Annane D, et al. Immunotherapy for Guillain-Barr syndrome: a systematic review. Brain. 2007 Sep;130(Pt 9):2245-57.
Van Koningsveld R, Steyerberg EW, Hughes RA, et al. A clinical prognostic scoring system for Guillain-Barr syndrome. Lancet Neurol. 2007 Jul;6(7):589-94.
Mori M, et al. Intravenous immunoglobulin therapy for Miller Fisher syndrome. Neurology. 2007 Apr 3;68(14):1144-6.