Download Guillain-Barré Syndrome

Page 1 of 4
View this article online at: patient.info/doctor/guillain-barre-syndrome-pro
Guillain-Barré Syndrome
Synonym: acute inflammatory polyneuritis
Guillain-Barré syndrome (GBS) is a disorder causing demyelination and axonal degeneration resulting in acute,
ascending and progressive neuropathy, characterised by weakness, paraesthesiae and hyporeflexia.
About 75% of patients have a history of preceding infection, usually of the respiratory and gastrointestinal tract. A
large number of infections have been linked, including Campylobacter jejuni, Epstein Barr virus, cytomegalovirus,
mycoplasma and human immunodeficiency virus [1, 2] . This association with preceding infection suggests that
antibodies to the infectious organism also attack antigens in peripheral nerve tissue.
The closest association between antibodies and the neurological disease is seen with the closely related Miller
Fisher syndrome (also called Fisher's syndrome), where more than 90% of patients have antibodies against the
ganglioside GQ1b. Miller Fisher syndrome is thought to be an inflammatory neuropathy affecting the cranial
nerves (especially the eye muscles, causing ophthalmoplegia), accompanied by areflexia and ataxia but not
weakness.
Bickerstaff's brainstem encephalitis is considered part of Miller Fisher syndrome with additional features of
drowsiness and extensor plantar responses. Some cases of acute inflammatory demyelinating
polyradiculoneuropathy (AIDP) have features of the Miller Fisher syndrome but with associated weakness [1] .
GBS consists of a number of subtypes, including [3] :
AIDP.
Acute motor and sensory axonal neuropathy (AMSAN): associated with the antibodies GM1, GM1b and
GD1a.
Acute motor axonal neuropathy (AMAN): associated with the antibodies GM1, GM1b, GD1a and
GalNac-GD1a.
Acute sensory neuronopathy: associated with GD1b antibodies.
Acute pandysautonomia.
About 95% of cases of GBS are AIDP.
Epidemiology
The incidence of typical GBS in Europe is 1.2-1.9 per 100,000 [3] .
There is an increased incidence in males. Peak ages are 15-35 years and 50-75 years.
Risk factors
History of gastrointestinal or respiratory infection from 1-3 weeks prior to the onset of weakness.
Recently, an association has been suggested with the Zika virus [4] .
Vaccinations: live and dead vaccines have been implicated.
Malignancies - eg, lymphomas, especially Hodgkin's disease.
Pregnancy: incidence decreases during pregnancy but increases in the months after delivery.
Page 2 of 4
Presentation
History
Weakness:
In 60% of cases, onset occurs approximately three weeks after a viral illness.
The condition usually presents with an ascending pattern of progressive symmetrical
weakness, starting in the lower extremities.
This reaches a level of maximum severity two weeks after initial onset of symptoms and
usually stops progressing after five weeks.
Facial weakness, dysphasia or dysarthria may develop.
In severe cases, muscle weakness may lead to respiratory failure.
Pain: neuropathic pain may develop, particularly in the legs. Back pain may be another feature.
Reflexes: these may be reduced or absent.
Sensory symptoms: these can include paraesthesiae and sensory loss, starting in the lower
extremities.
Autonomic symptoms: involvement of the autonomic system may present, with reduced sweating,
reduced heat tolerance, paralytic ileus and urinary hesitancy. Severe autonomic dysfunction may
occur.
Examination
The following features may be present:
Hypotonia.
Demonstrable altered sensation or numbness.
Reduced or absent reflexes.
Fasciculation may occasionally be noted.
Facial weakness - may be asymmetrical.
Autonomic dysfunction - fluctuations of heart rate and arrhythmias, labile blood pressure and variable
temperature.
Respiratory muscle paralysis.
Differential diagnosis
Other causes of acute paralysis include:
Brain: stroke, brainstem compression, encephalitis.
Spinal cord: cord compression, poliomyelitis, transverse myelitis.
Peripheral nerve: vasculitis, lead poisoning, porphyria.
Neuromuscular junction: myasthenia gravis, botulism.
Muscle: hypokalaemia, polymyositis.
Investigations
Diagnosis is usually made on clinical grounds. However, the following may be helpful:
Electrolytes: inappropriate antidiuretic hormone secretion occurs in some patients; serum and urine
osmolarity studies are indicated if it is suspected.
Lumbar puncture: most patients have an elevated level of cerebrospinal fluid (CSF) protein, with no
elevation in CSF cell counts. The rise in the CSF protein may not be seen until 1-2 weeks after the
onset of weakness.
Antibody screen: antibodies to peripheral and central nerves may be present.
Spirometry: forced vital capacity is a major determinant of the need for admission to ICU and then the
need for intubation.
Page 3 of 4
Nerve conduction studies: are the most useful confirmatory test and are abnormal in 85% of
patients, even early on in the disease. They should be repeated after two weeks if they are initially
normal [1] . A decrease to less than 20% of predicted normal is associated with a poorer prognosis.
ECG: many different abnormalities may be seen - eg, second-degree and third-degree AV block, Twave abnormalities, ST depression, QRS widening and a variety of rhythm disturbances.
Management
Plasma exchange [5] :
A Cochrane review found more improvement with plasma exchange than supportive care
alone, without a significant increase in serious adverse events. There was a small but
significant increase in the risk of relapse during the first 6-12 months after onset in people
treated with plasma exchange. However, after one year, full recovery was significantly more
likely and severe residual weakness less likely with plasma exchange.
Intravenous immunoglobulin [6] :
A Cochrane review found evidence that, in severe disease, intravenous immunoglobulin
started within two weeks from the onset hastens recovery as much as plasma exchange.
Intravenous immunoglobulin is significantly much more likely to be completed than plasma
exchange.
Giving intravenous immunoglobulin after plasma exchange did not confer significant extra
benefit.
Corticosteroids: according to moderate-quality evidence, corticosteroids given alone do not
significantly hasten recovery from GBS or affect the long-term outcome; they may even delay
recovery [7] .
Deep vein thrombosis (DVT) prophylaxis: DVT due to immobility should be prevented with gradient
compression stockings and subcutaneous low molecular weight heparin. See separate Prevention of
Venous Thromboembolism article.
Admission to the intensive care unit: intubation and assisted ventilation may be required.
Pain relief : may be required for neuropathic pain.
Complications
Possible complications include:
Persistent paralysis.
Respiratory failure requiring mechanical ventilation.
Hypotension or hypertension.
Thromboembolism, pneumonia, skin breakdown.
Cardiac arrhythmia.
Ileus.
Aspiration pneumonia.
Urinary retention.
Psychiatric problems - eg, depression, anxiety.
Prognosis
With modern intensive care support, the outcome is excellent for most patients. However, neurological
problems persist in up to 20% of patients; half of these patients are severely disabled [8] .
Approximately 20% of patients cannot walk unaided six months after the onset of the condition [9] .
The mortality rate is estimated to be 3-7% [9] . Death tends to be due to respiratory failure, pulmonary
emboli or infection.
Poor prognosis is associated with rapid progression of symptoms, advanced age and prolonged
ventilation.
High age, preceding diarrhoea, and degree of muscle dysfunction at hospital admission and at one
week are independently associated with being unable to walk at four weeks, three months and six
months [10] .
Page 4 of 4
Further reading & references
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
Winer JB; Guillain-Barre syndrome. BMJ. 2008 Jul 17;337:a671. doi: 10.1136/bmj.a671.
Kuwabara S; Guillain-Barre syndrome: epidemiology, pathophysiology and management. Drugs. 2004;64(6):597-610.
Hughes RA, Cornblath DR; Guillain-Barre syndrome. Lancet. 2005 Nov 5;366(9497):1653-66.
BlazquezAB, Saiz JC; Neurological manifestations of Zika virus infection. World J Virol. 2016 Nov 12;5(4):135-143.
Raphael JC, Chevret S, Hughes RA, et al; Plasma exchange for Guillain-Barre syndrome. Cochrane Database Syst Rev.
2012 Jul 11;7:CD001798. doi: 10.1002/14651858.CD001798.pub2.
Hughes RA, Swan AV, van Doorn PA; Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst
Rev. 2010 Jun 16;(6):CD002063.
Hughes RA, Brassington R, Gunn AA, et al; Corticosteroids for Guillain-Barre syndrome. Cochrane Database Syst Rev.
2016 Oct 24;10:CD001446.
Walling AD, Dickson G; Guillain-Barre syndrome. Am Fam Physician. 2013 Feb 1;87(3):191-7.
Willison HJ, Jacobs BC, van Doorn PA; Guillain-Barre syndrome. Lancet. 2016 Aug 13;388(10045):717-27. doi:
10.1016/S0140-6736(16)00339-1. Epub 2016 Mar 2.
Walgaard C, Lingsma HF, Ruts L, et al; Early recognition of poor prognosis in Guillain-Barre syndrome. Neurology. 2011
Mar 15;76(11):968-75. doi: 10.1212/WNL.0b013e3182104407.
Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical
conditions. EMIS has used all reasonable care in compiling the information but makes no warranty as to its
accuracy. Consult a doctor or other healthcare professional for diagnosis and treatment of medical conditions.
For details see our conditions.
Original Author:
Dr Colin Tidy
Current Version:
Dr Nick Imm
Peer Reviewer:
Dr John Cox
Document ID:
2213 (v25)
Last Checked:
06/12/2016
Next Review:
05/12/2021
View this article online at: patient.info/doctor/guillain-barre-syndrome-pro
Discuss Guillain-Barré Syndrome and find more trusted resources at Patient.
© Patient Platform Limited - All rights reserved.