Guillain-Barré Syndrome

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Guillain-Barré Syndrome (GBS)\: acute in
Aetiology\: 66% of cases follow an upper respiratory tract infection or gastrointestinal infection; associated organisms
include cytomegalovirus, Epstein-Barr virus, Mycoplasma, and Campylobacter jejuni.
Pathophysiology\: autoimmune condition involving molecular mimicry resulting in demyelination of motor and sensory
peripheral nerves; four subtypes\: AIDP, AMAN, AMSAN, and Miller-Fisher syndrome.
Symptoms\: rapidly progressive symmetrical weakness (ascending pattern), paraesthesia, balance issues, back/limb pain,
visual and speech di
Examination
Di
Investigations\: serial lung function tests, ECG, continuous BP monitoring, FBC, U&Es, LFTs, glucose, CK, nerve conduction
studies, lumbar puncture.
Management\: admit for assessment and monitoring; serial lung function tests, supportive treatment, VTE prophylaxis, eye
care, pressure sore management, pain relief, physiotherapy, swallow assessment; no bene
Further management\: plasma exchange, IV immunoglobulin therapy (IVIg), improved outcomes when started within two
weeks of symptom onset.
ICU admission indications\: signi
arrhythmias, swallow dysfunction.
Complications\: respiratory compromise, venous thromboembolism, infection, aspiration pneumonia, cardiac arrhythmias,
ileus, long-term disability, persistent neuropathic pain or fatigue, psychological trauma.
Prognosis\: 80% of patients regain ability to walk within six months; 20% have persistent neurological dysfunction; mortality
rate 3-10%.
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Introduction

Guillain-Barré Syndrome (GBS) is an acute, in
1
It is a relatively rare condition, with an incidence of approximately 2/100,000 every year in the UK. This number, however,
increases with age and is more prevalent in men than women.
2
While the full aetiology of Guillain-Barré Syndrome is not fully understood, two-thirds of patients who develop GBS have a
recent history of either an upper respiratory tract infection or gastroenteritis.
Established pharmacological therapies for GBS have shown improved patient outcomes, but management focuses on
reducing the risk of respiratory compromise, which is the major risk with GBS. Respiratory compromise occurs in
approximately 20-30% of GBS patients.
1Aetiology
Around 66% of GBS cases involve a previous history of either an upper respiratory tract infection (URTI) or a
gastrointestinal infection. 1
The most commonly-associated organisms are cytomegalovirus, Epstein-Barr virus,
M y c o p l a s m a , and C a m p y l o b a c t e r j e j u n i .
3
Guillain-Barré Syndrome is considered to be an autoimmune condition. Current evidence suggests GBS occurs due to
immune system activation against these pathogens, where the immune response mistakes key components of peripheral
nerves for the virus it is attacking. This phenomenon is termed molecular mimicry.
3
The result is demyelination of both motor and sensory peripheral nerves, impacting their ability to relay information
between the central nervous system and the periphery. This causes clinical features such as paraesthesia and weakness.
There have been reports of patients developing GBS following Zika virus outbreaks or more rarely COVID-19.
3
Pathophysiology
There are four subtypes of GBS\:
2
Acute in
North America, primarily a
Acute motor axonal neuropathy (AMAN)\: a form of GBS more common in Asian countries that primarily damages the
axons of motor neurons speci
sensorimotor neuropathy in AIDP.
Acute motor sensory axonal neuropathy (AMSAN)\: a
associated with a poorer rate of recovery.
Miller-Fisher syndrome\: a form of GBS de
ophthalmoplegia, ataxia, and are
A patient with GBS may exhibit characteristics of one or more variants. Identifying the subtype of GBS is important as they
vary as to the degree of respiratory involvement, and therefore the indication for early pharmacological therapies or
intensive care unit admission.
Figure 1. Patterns of symptom
distribution of di
Guillain-Barre Syndrome

Clinical features

History
GBS can present at any age, however, is more common in older men. A typical history may include recent viral infection or
travel abroad (with increased risk of C a m p y l o b a c t e r infection), and the
weeks post-infection.
2
Typical symptoms of GBS include\:
3
Rapidly progressive symmetrical weakness typically in an ascending pattern\: beginning in the lower limbs and hands
and starting to a
Paraesthesia (numbness and tingling) in the lower limbs and hands
Issues with balance or coordination, especially in the lower body
Back or limb pain which can be worse at night
Di
Di
There is variation in the progression of symptoms and can take hours, days, or weeks to resolve. Symptoms will progress
up to a maximum of 4 weeks, after which two-thirds of patients will recover and regain normal function within 6-12
months.
2
Clinical examination
A thorough neurological examination (including upper limbs, lower limbs and cranial nerves) should be carried out on all
patients suspected of Guillain-Barré Syndrome.
Typical clinical
4
Symmetrical bilateral weakness ascending from the lower limbs
Reduced sensation over areas of weakness (such as legs and hands)
Are
Autonomic dysfunction\: heart arrhythmias, tachycardia, hyper- or hypotension, anhidrosis, respiratory dysfunction

Di

Di
4
Stroke
Encephalitis
Myasthenia gravis
Polymyositis
Myelopathy
Botulism

Investigations

Guillain-Barré Syndrome is a clinical diagnosis. However, investigations help stratify risk in patients. Due to the rare and
complex nature of GBS, all suspected cases should be referred to hospital for specialist review and possible admission.
Bedside investigations
Relevant bedside investigations include\:
4
Serial lung function tests\: the greatest risk in a patient with GBS is the threat of respiratory compromise, as more
muscles are at risk of paralysis as the condition progresses. Patients should have regular and frequent measurements of
their Forced Vital Capacity (FVC). Any patients with an FVC \<50% predicted or with rapid deterioration should be
reviewed for intubation and mechanical ventilation.
Electrocardiogram (ECG)\: to screen for possible heart arrhythmias and heart block
Continuous BP monitoring (in severe cases)\: GBS can cause autonomic dysfunction. Continuous monitoring would often
take place in an ICU environment.
Figure 2. Handheld spirometry device
Laboratory investigations
Relevant laboratory investigations include\:
2
Full blood count\: for baselineUrea and electrolytes\: up to 50% of patients may have syndrome inappropriate ADH secretion (SIADH), the mechanism
is unclear, but this may be a side e
symptoms and should be excluded.
Liver function tests\: often associated with mildly raised ALT and AST
Glucose\: hypoglycemia can mimic GBS symptoms
Creatine kinase (CK)\: to exclude a polymyositis
Other specialist immunological blood tests may be performed, speci
Fisher syndrome.
Imaging
There are no de
specialists may request ultrasound imaging of peripheral nerves.
Special tests
Other relevant investigations include\:
5
Nerve conduction studies\: shows the rate at which electrical signals travel across the nerves. In GBS, due to the di
polyneuropathy a
(though it may be normal in early disease). This is considered the gold standard investigation if there is diagnostic
uncertainty.
Lumbar puncture\: often shows CSF containing increased protein and normal cell count. This is usually not seen in the

Management

In the acute phase of Guillain-Barré Syndrome, there is a risk of rapid deterioration. As such, all patients should be
admitted for assessment and close monitoring.
General management
General management of GBS includes\:
2
Serial lung function tests (FVC)\: every four hours to screen for respiratory compromise
Supportive treatment\: intravenous
Venous thromboembolism (VTE) prophylaxis
Eye care
Pressure sore screening and management
Pain relief\: neuropathic pain medications such as amitriptyline or gabapentin. May require opiates for severe pain.
Physiotherapy\: to help regain motor function and avoid contractures
Swallow assessment\: patients may require nasogastric feeding if dysphagia is present
Corticosteroids, often used for other classical autoimmune conditions, have shown no clinical bene
Further management
There are two current therapies designed to minimise the autoimmune damage being done to the nerves\:
5
Plasma exchange\: involves plasma within the blood being removed,
circulation around the body. This decreases the severity of GBS by removing the autoantibodies within the blood
damaging the nerves.
IV immunoglobulin therapy (IVIg)\: an infusion of donor immunoglobulins (antibodies) which dilutes the ability of the
autoantibodies to in
Both of these therapies show improved clinical outcomes for patients when started within two weeks of symptom onset.
Almost all patients admitted with Guillain-Barre syndrome (apart from those with Miller-Fisher syndrome) will be treated
with plasma exchange or intravenous immunoglobulin.Admission to intensive care
Indications for intensive care unit admission in severe GBS include\:
4
Requirement of signi
Rapidly-progressing muscle weakness
Rapidly
Swallow dysfunction, with the risk of aspiration pneumonia
The Erasmus GBS Respiratory Insu can help predict the risk of respiratory compromise in the

6

Complications

Complications of Guillain-Barré syndrome may include\:
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Respiratory compromise (the leading cause of death in GBS patients)
Venous thromboembolism
Infection
Aspiration pneumonia
Cardiac arrhythmias
Ileus
Long-term disability, requiring a wheelchair or walking aid
Persistent neuropathic pain or fatigue
Psychological trauma of experience causing lasting disability and distress
The general prognosis is good with 80% of patients regaining the ability to walk six months following the course of the
disease. However, 20% of patients have persistent neurological dysfunction. There is a mortality rate of 3-10%.
4

References

BMJ Best Medical Practice. G u i l l a i n-B a r r e S y n d r o m e . Last reviewed 19 Aug 2022. Available from\: [LINK]
Lo
from\: [LINK]
National Institute of Neurological Disorders and Stroke. G u i l l a i n-B a r r é S y n d r o m e F a c t S h e e t . Published June 2018. Available
from\: [LINK]
Shaf, Suchita. Patient UK. G u i l l a i n-B a r r e S y n d r o m e . Last edited 14 July 2022. Available from\: [LINK]
National Organization for Rare Diseases. G u i l l a i n-B a r r e S y n d r o m e . Published 2020. Available from\: [LINK]
Walgaard, C., Lingsma, H.F., et al. Annals of Neurology 2010. P r e d i c t i o n o f r e s p i r a t o r y i n s u
s y n d r o m e , Published 25 May 2010. Available from\: [LINK]
Image References
Figure 1. Leonhard, S.E., Mandarakas, M.R., Gondim, F.A.A. et al. S y m p t o m d i s t r i b u t i o n o f d i
S y n d r o m e . License\: [CC BY 4.0]
Figure 2. Cosmed. D e s k t o p s p i r o m e t e r ( c r o p p e d ) . License\: [CC BY-SA 3.0]

Reviewer

Dr James Miller
Consultant NeurologistRoyal Victoria In

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Contents

Introduction
Aetiology
Clinical features
Source\: geekymedics.com