Guillain-Barré Syndrome - ClinicalKey

CLINICAL OVERVIEW
Guillain-Barré Syndrome
Elsevier Point of Care (see details)
Updated April 6, 2022. Copyright Elsevier BV. All rights reserved.
Synopsis
Urgent Action
Hospitalize all patients with suspected Guillain-Barré syndrome. There is a low
threshold for placement in the ICU 1
Intubate or support patients who have respiratory failure with mechanical

ventilation 2
Surgically insert a pacemaker in patients with symptomatic bradycardia 2
Key Points
Guillain-Barré syndrome is an acute, progressive, monophasic paralytic neuropathy
resulting from aberrant autoimmunity, typically in response to a causative infection
Campylobacter jejuni is the most common precursory infection

Diagnosis is made based on clinical signs and symptoms, especially the combination of
hyporeflexia and symmetrical ascending weakness peaking in 4 weeks or less 1
Miller Fisher syndrome, a rare subtype, is associated with ataxia and ophthalmoplegia
Albuminocytologic dissociation, consisting of high levels of protein in the cerebrospinal

fluid without an increase in cell counts, is characteristic
EMG studies, which typically demonstrate demyelinating features, support the diagnosis
Treatment involves immediate hospitalization, immunotherapy with plasma exchange or IV
immunoglobulin, supportive care, close monitoring for respiratory involvement (which may
require mechanical ventilation), and monitoring for dysautonomia (which may require
hemodynamic stabilization)
During the progressive phase, autonomic dysfunction manifests in two-thirds of patients,

causing ileus, causing arrhythmia, and/or contributing to respiratory failure 1
Pneumonia, sepsis, pulmonary embolism, and gastrointestinal bleeding develop in up to

60% of intubated patients during acute (progressive) disease 2
Most patients (65%) have a good prognosis for recovery. In developed countries, 5% of
patients with Guillain-Barré syndrome die from complications 3
Long-term complications may include severe fatigue (80% 2), residual pain (33% 3), and
inability to walk unaided (20% of severely affected patients 4)
Pitfalls
Without close and attentive monitoring, rapid deterioration of the patient's condition may
be missed
Pain and severe fatigue associated with the disease are often overlooked or undertreated
Terminology
Clinical Clarification
Guillain-Barré syndrome is an acute, progressive, monophasic paralytic neuropathy
resulting from an autoimmune response affecting the peripheral nerves and their spinal
roots, typically in the wake of immune stimulation by an infectious disease 1
Campylobacter jejuni is the causative pathogen in 25% to 50% of adult patients 1

Albuminocytologic dissociation 3
Consists of high levels of protein in the cerebrospinal fluid without an increase in cell
counts
Is present in 50% of patients in the first week and 75% of patients by the third week
after symptom onset
Classification
Guillain-Barré syndrome can be classified as follows:
Acute inflammatory demyelinating polyneuropathy

Immune injury occurs specifically at the myelin sheath and associated Schwann cell
components, causing vesicular degeneration of the Schwann cells 1
Facial diplegia with paresthesias is a localized form of this subtype 3
Most common subtype in Europe and North America, excluding Mexico (90% of cases)
3
Second most common subtype in China, Japan, Bangladesh, and Mexico (22%-46% of
cases)
Acute motor axonal neuropathy
Immune injury occurs primarily to the axolemma itself, leading to nerve conduction
failure 1
Pharyngeal-cervical-brachial weakness—marked by acute weakness of the

oropharyngeal, neck, and shoulder muscles—is a localized form of this subtype 3
Most common subtype in China, Japan, Bangladesh, and Mexico (30%-65% of cases) 3
There was a significant increase in this variant of Guillain-Barré syndrome during

the summers of 1991 and 1992 in a rural area of China 4
This form is sometimes referred to as Chinese paralytic syndrome 4
Second most common subtype in Western countries (5%-10% of cases 6) 5
Acute motor-sensory axonal neuropathy 6
Rare subtype with more pronounced sensorial symptoms
Accounts for 5% to 10% of cases in the United States and Europe 4
Miller Fisher syndrome
Characterized by clinical symptoms relating to dysfunction of the third, fourth, and

sixth cranial nerves 7
Typically presents as ophthalmoplegia with ataxia and areflexia and as associated distal
paresthesia without weakness
Accounts for 5% of cases in Western countries 5
Diagnosis
Clinical Presentation
History
Common symptoms in the prodromal period of Guillain-Barré syndrome are fever (52%
of patients), cough (48%), sore throat (39%), rhinorrhea (30%), and diarrhea (27%) 8
Fever due to a causative infection is significantly associated with the presence of bulbar
palsy (affecting cranial nerves IX, X, XI, and XII) causing dysphagia, dysphonia, and
dysarthria
Patients with sore throat or cough often develop ophthalmoplegia and headache with
facial palsy
Patients with diarrhea rarely show ophthalmoplegia or bulbar palsy
Diarrhea and abdominal pain are closely linked to Campylobacter jejuni cause
About two-thirds of patients had symptoms of an infection in the 3 weeks before onset
of weakness 1
Characteristic symptom is progressive weakness of more than 2 limbs, with progression

lasting fewer than 4 weeks 7
Weakness is classically described as ascending and symmetrical, typically manifesting
first in the following: 1
Lower limbs as both proximal and distal weakness (56%) 5
Legs and arms simultaneously (32%) 5
Upper limbs (12%) 5
Typical course is acute progression of limb weakness, beginning as early as 12 hours

after onset and proceeding to its maximum clinical deficit in 2 to 4 weeks 1
Sensory and cranial nerve involvement often occurs 1 to 2 weeks after infection or
other immune stimulation 1
Accompanying facial weakness is typically bilateral 1
Disease nadir occurs within 2 weeks after infection in 80% of patients, in fewer than
4 weeks in 97%, and in fewer than 6 weeks in all patients 9
Respiratory muscle weakness tends to progress slowly and to be roughly correlated

with the degree of extremity muscle weakness 1
Inability to take a deep breath, associated with respiratory failure, has been reported
in 20% to 30% of patients
Infants present with irritability, decreased movement, and hypotonia; they may present
with respiratory insufficiency 10
Older children present with fatigue and weakness and have trouble walking, climbing
stairs, and rising from the floor; at the disease nadir, approximately 60% of children
are not be able to walk and 24% are not able to move their arms 10
Pain precedes weakness in one-third of adult patients with Guillain-Barré syndrome 3
Two-thirds of all adult patients experience pain during the acute phase of the disease,
especially back pain; 3 for approximately 50% of these, the pain is severe 2
Pain may manifest as dysesthesia or muscular, radicular, or arthralgic pain; pain is

worse with movement
Pain is the initial complaint in up to 50% of children 10
Symptoms of autonomic dysfunction include the following:
Orthostasis 4
Syncope or near-syncope 1
Urinary retention
Guillain-Barré syndrome may be the first symptom of HIV infection when triggered by
this condition 11
Acute motor axonal neuropathy
Similar to acute inflammatory demyelinating polyneuropathy with these exceptions:
Abrupt onset of motor weakness and acute flaccid paralysis
Neuropathy is purely motor, although 10% of patients report distal paresthesias 12

Cranial nerve involvement occurs less frequently than in demyelinating
polyneuropathy
Autonomic dysfunction is not present or is mild
Acute motor-sensory axonal neuropathy
Similar to acute inflammatory demyelinating polyneuropathy with more sensory

involvement; it is difficult to differentiate the two without neurophysiologic studies
Has a more severe clinical course than acute inflammatory demyelinating

polyneuropathy
Miller Fisher syndrome

Associated symptoms include blurred vision and dysarthria
Patients typically present with cranial nerve involvement resulting in facial, oculomotor,
or bulbar weakness, which may extend to the limbs
Distal paresthesia is closely associated with this variant
Physical examination
Bilateral symmetrical flaccid muscular weakness; typically begins in distal legs and
rapidly progresses toward the oral and nasal region
In children, weakness initially may be asymmetrical 10
There is occasional bilateral involvement of facial, oropharyngeal, and oculomotor

muscles
Hyporeflexia or areflexia is a hallmark of Guillain-Barré syndrome (acute inflammatory

demyelinating polyneuropathy) 3
Decreased tendon reflexes are common 1
10% of patients with acute motor axonal neuropathy have normal or brisk reflexes
during the course of illness 3
Children usually lose reflexes during the first week of illness 10
Sensory examination in adults reveals mild decrease in touch sensation in most patients
Approximately 40% of children have loss of sensation (usually distal) 10
Facial paresis occurs in more than 50% of patients, usually bilateral 13

Signs of autonomic dysfunction (usually occurring in advanced or severe disease) include:
Extreme hypertension or hypotension; occurs in 20% of patients 3
Wide variation (greater than 85 mm Hg) in systolic blood pressure from day to day 3
Arrhythmias ranging from bradycardia to tachycardia
Bradycardia may be severe, possibly causing asystole 3
Abnormal pupillary response to light 1
Acute motor axonal neuropathy 14
Motor findings as per acute inflammatory demyelinating polyneuropathy except more

rapid progression in acute motor axonal neuropathy
Sensory examination is normal
Patient usually has preserved normal reflexes, with hyperreflexia at the peak of the illness:
10% have normal or exaggerated reflexes throughout the illness
5% have intact reflexes on presentation but become areflexic as the disease progresses
Acute motor-sensory axonal neuropathy
Presents as acute inflammatory demyelinating polyneuropathy with symmetrical

muscular weakness (often a profound quadriparesis), decrease in peripheral sensation,
and areflexia 15
Rapidly progresses, with maximal deficit observed by day 7
Miller Fisher syndrome 1
Characterized by the triad of ophthalmoplegia, ataxia, and areflexia
Corneal reflex may be compromised 16
Causes and Risk Factors
Causes
Guillain-Barré syndrome is typically preceded by infection or other immune stimulation
that induces an aberrant autoimmune response, targeting peripheral nerves and their spinal
roots 1
Two-thirds of cases are preceded by symptoms of upper respiratory tract infection or
diarrhea 1
Autoimmunity does not arise in 99% of people exposed to Guillain-Barré syndrome–

associated infections 1
Causative infections associated with the pathogenesis of Guillain-Barré syndrome
Campylobacter jejuni infection is identified in up to 50% of patients 1

Associated with axonal Guillain-Barré syndrome (acute motor axonal neuropathy)
and Miller Fisher syndrome 7
Cytomegalovirus is identified in 10% of patients 3
Associated with demyelinating Guillain-Barré syndrome (acute inflammatory

demyelinating polyneuropathy) 7
Epstein-Barr virus, Influenza A virus, Mycoplasma pneumoniae, Haemophilus

influenzae, Zika virus, and Chikungunya virus 1
Epstein-Barr virus is associated with acute inflammatory demyelinating
polyneuropathy 7
Molecular mimicry between microbial and nerve antigens is highly significant,

especially in the case of Campylobacter jejuni infection 1
Both cell-mediated and humoral mechanisms are implicated in pathogenesis 7
Immunologic studies suggest that more than one-third of patients generate

antibodies against nerve gangliosides 7
95% of those with Miller Fisher syndrome carry these antibodies 7
HIV-associated Guillain-Barré syndrome has been observed; this neuropathy generally

occurs early in HIV infection, even at seroconversion 11
Guillain-Barré syndrome has been reported shortly after vaccination with Semple rabies
vaccine and different strains of Influenza A virus vaccine (rare) 1
Risk factors and/or associations
Age
Incidence rises by 20% for every 10-year increase in age 17
Age-specific rate increases from 0.62 (cases per 100,000 person-years) among children
aged 0 to 9 years to 2.66 among adults aged 80 to 89 years 17
Sex
More prevalent in males; elevated relative risk of 1.78 17
Genetics
Genetic contributors are suspected, especially genes that govern immune response and
nervous system effects; 18 Guillain-Barré syndrome has been observed in at least 2 members
of some families 19
Other risk factors/associations

Use of drugs such as heroin, suramin, streptokinase, and isotretinoin 20
Tumor necrosis factor-α antagonist therapy 21
Autoimmune disorders such as systemic lupus erythematosus
Procedures and conditions that have been associated with Guillain-Barré syndrome include
surgery, epidural anesthesia, bone marrow transplant, immunizations, and concurrent
illnesses such as Hodgkin disease and sarcoidosis 22
A potential association between SARS-CoV-2 (COVID-19) and Guillain-Barré syndrome has

been reported 23 24 25
Diagnostic Procedures
Primary diagnostic tools

Patient history and physical examination findings are crucial to the diagnosis 1
The following Brighton criteria have been validated for diagnosis of Guillain-Barré
syndrome: 9
Bilateral and flaccid weakness of limbs (100% of patients)
Decreased or absent deep tendon reflexes in weak limbs (91% of patients)
Monophasic course and time between onset and nadir is 12 hours to 28 days (97%
of patients)
Cerebrospinal fluid cell count fewer than 50/μL (100% of patients)

Cerebrospinal fluid protein concentration greater than reference range (49% of
patients initially, 88% at 3 weeks)
Nerve conduction study results consistent with 1 of the Guillain-Barré syndrome

subtypes (99% of patients)
Absence of alternative diagnosis for weakness
Brighton criteria help establish the level of diagnostic certainty based on the clinical
signs, disease course, and test results 9
The more Brighton criteria identified, the greater the likelihood of Guillain-Barré
syndrome 9
Ranges from level 1 (highest level of diagnostic certainty, with all criteria met) to
level 4 (diagnosed as Guillain-Barré syndrome, possibly due to equivocal findings
or insufficient data for further classification)
Perform a lumbar puncture in all patients when Guillain-Barré is suspected 1
Albuminocytologic dissociation (combination of a normal cell count and raised

protein level in the spinal fluid) is a classic sign, found in approximately 90% of
patients at peak disease, with these caveats: 1
Found in only half of patients on initial analysis 9
Normal protein level (especially when measured in week 1 after disease onset)
does not exclude the diagnosis; 10% of patients with Guillain-Barré syndrome
have normal cerebrospinal fluid protein analysis 5
15% of patients with the disease do show a mild increase in cerebrospinal fluid cell
count (5-50 cells/μL) 1
If HIV is also present, pleocytosis is not an aberration but the norm 26
Electrodiagnostic nerve studies support the diagnosis and provide prognostic

information 1
Perform in all patients when available
Can confirm the presence, pattern, and severity of neuropathy
Features of acquired demyelination are characteristic of acute inflammatory

demyelinating polyneuropathy
MRI scans of the spine may be as useful as electrodiagnostic studies in supporting the
diagnosis, especially in pediatric patients 27
MRI may be of particular benefit when specialist neurophysiologist consultation is
unavailable
Laboratory
Imaging
Functional testing
Procedures
Differential Diagnosis
Most common
Acute-onset chronic inflammatory demyelinating neuropathy 32
Symmetrical peripheral sensory neuropathy and weakness caused by nerve fiber

demyelination; autonomic dysfunction is not a feature of chronic inflammatory
demyelinating neuropathy
In 15% of patients, there is acute onset of neuropathy; the plateau is reached within 4
weeks, similar to Guillain-Barré syndrome 32
Subsequent relapsing or progressive course of disease with cranial nerve involvement

occurs in 16% of patients; the course of Guillain-Barré syndrome is monophasic 32
Diagnosis is usually made clinically with support provided by the following:
Electrophysiological studies demonstrating impaired sensory and motor conduction
Motor nerve conduction velocities slower than 40 m/second in median and ulnar
nerves, or slower than 30 m/second in the peroneal nerve 32
Terminal latency greater than 7 milliseconds in the median or ulnar nerves or 10

milliseconds in the peroneal nerve 32
Conduction block and/or differential dispersion of the compound muscle action

potential
Sural nerve biopsy showing more than 15% teased demyelinated fibers 32
Lyme disease 33 (Related: Lyme Disease)

Neuropathic pain, facial palsy, and other cranial neuropathies may be present in
advanced, untreated disease
Neuropathy is often more asymmetrical than that of patients with Guillain-Barré

syndrome
Classic initial manifestation is erythema migrans, also known as a bull's-eye rash, growing
to a diameter of 5 to 30 cm in 80% of patients 33
Confirm diagnosis by serologic testing (eg, sensitive enzyme immunoassay,

immunofluorescent assay test) 34
For all positive or equivocal specimens, use a Western blot to test for both IgG and IgM
antibodies to Borrelia bacterial antigen. (In 2019, the FDA cleared several Lyme disease
serologic assays with new indications for use, allowing for an enzyme immunoassay
rather than Western immunoblot assay as the second test.)
Acute hepatic porphyrias, including acute intermittent porphyria 35
Porphyrias caused by deficiencies in the activities of heme biosynthetic pathway enzymes
Peripheral neuropathy with motor weakness may be present in acute hepatic porphyrias,
as well as cranial nerve involvement 36
These porphyrias are inherited and not precipitated by another illness; abdominal pain is
typically present
Proximal muscles are predominantly affected in 80% of patients, with onset in the
upper limbs in 50% 36
Confirm diagnosis by noting elevated levels of porphobilinogen in urine or plasma
Vasculitic neuropathy 37
Inflammation or occlusion of blood vessels that can lead to ischemic peripheral

neuropathy
Peroneal and ulnar nerves are commonly involved
Isolated cases may resemble the course of Guillain-Barré syndrome, with rapid onset of
distal peripheral neuropathy
Typically much more painful than Guillain-Barré syndrome
Women are affected more often than men (nearly 60% versus 40%), and average age at
diagnosis is approximately 60 years 37
Confirm diagnosis by histopathology of a sural nerve or superficial peroneal nerve biopsy
and concomitant biopsy of the gastrocnemius or peroneus brevis muscle; both will reveal
vascular inflammatory lesions with features including:
Transmural vessel wall infiltration; leukocytoclasis; destruction of the endothelium,

internal elastic lamina, or smooth muscle cells in tunica media; perivascular and
vascular hemorrhage; or occlusion of vessel lumen
Hyperkalemic periodic paralysis 38
Autosomal dominant muscle channelopathy with periodic focal weakness, often involving
thigh and calf muscles (rarely, facial or respiratory muscles) in response to rising serum
potassium levels (either at least a 1.5 mmol/L increase or a concentration above 5 mmol/L
[5 mEq/L])
Attacks may be precipitated by potassium loading, anesthesia, rest after exercise,

hunger, change in activity level, cold, menstruation, emotional stress, pregnancy,
illness, or glucocorticoids
Onset begins before age 20 years
Attacks can last longer than 1 week
Diagnosed by history; serum potassium level may be within reference range or elevated
during an attack
Hypokalemic periodic paralysis 39
Acute flaccid paralysis when serum potassium drops below 3.5 mEq/L
May be due to a single gene mutation affecting potassium, sodium, or calcium

channels or secondary to hyperthyroidism (acute thyrotoxicosis), renal or
gastrointestinal loss, or diuretic or other drug use
Paralysis may resemble Guillain-Barré syndrome
Ascending paralysis can develop, with eventual impairment of respiratory function
Early morning symptoms (eg, paralysis occurring in the morning) and weakness
following a meal or strenuous exercise occurs in almost 50% of patients
Diagnosis can be confirmed by serum measurement of potassium and resolution of

symptoms with correction of hypokalemia
Acute cervical myelopathy due to transverse myelitis or cord compression
Bilateral spinal cord dysfunction developing over approximately 4 weeks with a well-
defined upper sensory level and no prior illness defines transverse myelitis or a
developing compression of the spinal cord 40
Also may present as a rapidly progressive paresis or paraplegia, starting with the legs
and sometimes progressing to involve the upper extremities
Initially, reflexes may be lessened or absent only to become hyperreflexic after a mean
duration of 4 to 6 weeks
Differentiating features include the following:
Urinary urgency or retention is a typical early finding of myelopathy, which is less

common in Guillain-Barré syndrome
In Guillain-Barré syndrome, dysesthetic pain, involvement of the upper extremities

and cranial nerves, and absent deep tendon reflexes are more likely
Diagnosis of myelopathy can be confirmed by clinical presentation and evidence of acute

inflammation on spinal MRI or spinal cord compressive lesion 40
Ocular myasthenia gravis (Related: Myasthenia Gravis)
Myasthenia gravis is an antibody-mediated autoimmune disorder of the neuromuscular

junction; ocular weakness presents in 85% of patients with myasthenia gravis as
fluctuating ptosis and/or diplopia 41
Ophthalmoplegia and autoimmune origin are characteristic of Miller Fisher syndrome as

well 42
Differentiating characteristics include the following: 42
Most patients with Miller Fisher syndrome can identify an infection preceding
symptoms
Ocular myasthenia gravis has a subacute or chronic onset with fluctuations in muscle
weakness; reflexes are present 41
Diagnosis is confirmed by the following: 41
Abnormal EMG results of the repetitive nerve stimulation test, typically in a proximal
or facial muscle, are characteristic of ocular myasthenia gravis
Repetitive nerve stimulation test result is abnormal in approximately 75% of

patients with generalized myasthenia gravis but less than 50% in patients with the
ocular form of disease 41
Serologic testing for acetylcholine receptor antibodies in ocular myasthenia gravis
Treatment
Goals
Diminish functional impact of disease
Manage pain
Prevent or reduce the impact of complications
Support recovery of function
Disposition
Admission criteria
All patients are admitted for supportive care 3
Criteria for ICU admission

Adults 3
ICU admission and mechanical ventilation are recommended in patients with at least 1
major criterion or 2 minor criteria:
Major criteria
Hypercapnia (PaCO₂ above 48 mm Hg)
Hypoxemia (PaO₂ below 56 mm Hg while the patient is breathing ambient air)
Vital capacity less than 15 mL/kg of body weight
Negative inspiratory force less than −30 cm H₂O
Minor criteria
Inefficient cough
Impaired swallowing
Atelectasis
Children 10
Pediatric ICU admission for any of the following:
Rapidly progressive course
Inability to flex neck or arms
Flaccid weakness of limbs
Significant bulbar, respiratory, or autonomic dysfunction
Intubation and mechanical ventilation is recommended for any of the following:
Reduction in vital capacity to less than 15 mL/kg body weight
PaO₂ is below 70 mm Hg
Respiratory fatigue
Recommendations for specialist referral

Refer all patients suspected of having Guillain-Barré syndrome to neurologist
Respiratory (ventilatory) failure and need for mechanical ventilation may require
consultation with an intensivist or a pulmonologist
Refer all patients to physical therapist early in the treatment course
Order speech-language evaluation for swallowing assessment and potential therapy
Treatment Options
The mainstay of treatment of Guillain-Barré syndrome remains adequate supportive care,
respiratory support when required, and immunotherapy 7
IV immunoglobulin and plasma exchange are both effective treatments for Guillain-Barré
syndrome 1 43
No difference exists between the 2 treatments in regard to improvement in disability grade

at 4 weeks, mechanical ventilation duration, residual disability, or mortality 44
Plasma exchange is effective when given within 2 weeks of illness onset in patients who are
unable to walk; it is most effective when given within 7 days of weakness onset 44
Adults
Immediately treat patients who cannot walk unaided with 1 of the following: 1
5 plasma exchange sessions over 2 weeks, with a total exchange of 5 plasma volumes 45
IV immunoglobulin daily for 5 days 1
IV immunoglobulin and plasma exchange are equally effective 46, but do not perform
both sequentially because there is no significant advantage 45
Patients who are able to walk (but not run) may benefit from 2 plasma exchanges of 1.5
plasma volumes 45
Treat with IV immunoglobulin those patients who fit the following descriptions who have
maintained the ability to walk (benefit has not been demonstrated consistently): 44
Mildly affected
Rapidly progressive limb weakness
Respiratory impairment
Severe swallowing difficulties
Autonomic dysfunction
Consider giving a second course of IV immunoglobulin to patients who deteriorate after
initial stabilization or improvement (ie, those in whom benefit has not been demonstrated
consistently) 3
Patients with Miller Fisher syndrome do not require immunotherapy, presumably because
they have good natural recovery 7 44
Consider IV immunoglobulin for patients with severe Miller Fisher syndrome who have
swallowing and respiratory difficulties, despite lack of evidence of benefit
Overall, IV immunoglobulin has been adopted widely instead of plasma exchange owing to
its convenience, wide availability, and minor adverse effects 1
Disadvantage of IV immunoglobulin is extremely high cost, which decreases the chance that
it can be used by low-income patients, in low-income areas, and by patients who are
underinsured 1
Children 10
IV immunoglobulin typically is preferred for children as it is easy to administer and has a

good adverse effect profile
Clinical response is noticeable in 3 to 7 days

Plasma exchange is safe and effective in children who weigh more than 10 kg
Perform exchange 4 to 6 times on alternate days to a total exchange of 250 mL/kg
One study indicated that plasma exchange may be superior to IV immunoglobulin

therapy for children who are severely affected and require ventilatory support
In severe cases and in patients who deteriorate after initial improvement (ie, treatment-
related fluctuation), consider trying to repeat courses of plasma exchange or IV
immunoglobulin
Corticosteroid therapy, previously used widely to treat Guillain-Barré syndrome, has been
shown to be ineffective both alone and in combination with IV immunoglobulin 46 47
Corticosteroids actually may slow recovery from Guillain-Barré syndrome, but they may
have a place in treatment of severe neuropathic or radicular pain
Mechanical ventilation is required for the 20% to 30% of patients who develop respiratory
failure; endotracheal intubation/tracheostomy may be necessary 1
Respiratory muscle fatigue is indicated by tachycardia, diaphoresis, tachypnea,

asynchronous chest and abdomen movement, and use of accessory muscles 2
If possible, postpone placing a tracheostomy for 2 weeks. After 2 weeks, if there is no

significant improvement in pulmonary function test results, tracheostomy is indicated; if
there is slight improvement, consider waiting one more week 2
Provide pain relief for acute pain that is both nociceptive and neuropathic in origin; a
combination of medications is typically used together owing to the mixed nature of the pain 48
Use opioids cautiously 4 as they can suppress respiratory drive, worsen autonomic gut
dysmotility, and increase urinary retention, which are often present in patients with
Guillain-Barré syndrome 2
Start therapy for neuropathic pain early in the course of the disease owing to the delayed
effect of the medications 2
Indicated medications include gabapentin, pregabalin, carbamazepine, and amitriptyline
Use corticosteroids, if necessary, to address neuropathic or radicular pain
Provide prophylactic therapy for deep vein thrombosis or pulmonary embolism risk by
administering subcutaneous heparin or enoxaparin and applying compression stockings for
adult patients who cannot walk 1
Manage effects of dysautonomia 2
Most complications related to dysautonomia occur in patients with respiratory failure and
advanced generalized weakness
Treatments include the following:
Nasogastric tube to allow feeding
Bladder catheterization to relieve urinary retention
Laxatives to relieve constipation
Management of hyponatremia
Syndrome of inappropriate antidiuretic hormone secretion develops in 21% 50 to 48%

51 of Guillain-Barré syndrome patients and is predictive of a poor outcome 49
Bradycardia leading to asystole may require a cardiac pacemaker; 2 pacemaker is rarely

required in children 10
Begin physical therapy early during the course of illness and start rehabilitation when
improvement is observed 1
Prevent contractures and stasis ulcers
In patients with facial weakness, prevent corneal ulceration (eg, use lubricant eye drops) 2
Drug therapy
IV immunoglobulin therapy
Before IV immunoglobulin therapy, check IgA levels because patients with IgA deficiency
(due to IgA antibodies) are at higher risk of anaphylaxis 26
Main component of immunoglobulin therapy is IgG, and the usual dose is 2 g/kg divided
over 2 to 5 days 52
If the increase in IgG is less than 10.92 g/L, consider a second session 52
Calculate change in IgG by subtracting the baseline level from the level at 2 weeks 52
Infuse IV immunoglobulin in patients with renal dysfunction at 50% of the normal rate
26
Immune Globulin (Human) Solution for injection; Children and Adolescents: 1

g/kg/dose IV daily for 2 days. Alternatively, 400 mg/kg/dose IV daily for 5 days has been
used.
Immune Globulin (Human) Solution for injection; Adults: In one study, patients
received IVIG 400 mg/kg IV daily for 5 days.
Anticoagulants
Heparin
Heparin Sodium (Porcine) Solution for injection; Adults: 5000 units subcutaneously
every 12 hours. 2
Enoxaparin
Enoxaparin Sodium (Porcine) Solution for injection; Adults: 40 mg subcutaneous daily.

2
Opioid analgesics
Possible adverse effects include suppression of the respiratory drive and worsening of
autonomic gut dysmotility and urinary retention; use these drugs with caution 4
In a study of pain intervention in patients with Guillain-Barré syndrome, 75% of
patients required oral or parenteral opioids for pain management and 30% of patients
required IV morphine (1-7 mg/hour) 2
Epidural morphine infusions (1-4 mg bolus injections every 8-24 hours) have been
used successfully 2
Nondrug and supportive care

Prophylaxis of venous thromboembolism 3
Support stockings reduce risk by almost 70% in adult patients at moderate risk 2
Deep vein thrombosis is very unlikely in children, hence prophylactic steps generally are not
taken
Physical therapy
Acute phase therapy includes gentle strengthening using isometric, isotonic, isokinetic, and
manual-resistive and progressive-resistive exercises 2
Rehabilitation will address proper limb positioning, nutrition, orthotics, and posture 2
Immunizations 2
Not recommended during the progressive phase of illness and not recommended for up to
1 year after illness
If Guillain-Barré syndrome develops within 6 weeks of an immunization, consider not

using that immunization again in that patient
Procedures
Plasma exchange therapy 45
General explanation
Removal of large-molecular-weight substances, including harmful antibodies, from plasma;
typically exchange with albumin (preferred over fresh frozen plasma)
5 plasma exchange sessions (each exchange comprising 2-3 L of plasma according to body
weight) over 2 weeks is the standard course for patients who are unable to walk unaided 1
Mildly affected patients (ie, Guillain-Barré syndrome disability score of 1-2) who are still
able to walk may benefit from 2 plasma exchanges of 1.5 plasma volumes 1
Plasma exchange and IV immunoglobulin therapy are considered to be equally effective 46
Indication
Standard treatment for patients with Guillain-Barré syndrome who are unable to walk
unaided 45
Also a treatment option for patients who are mildly affected and still able to walk 1
Contraindications
Prior use of IV immunoglobulin infusion therapy 45
Hemodynamically unstable patients 1
Pregnant patients 1
Sepsis
Hypocalcemia
Complications 53
Hematomas at venipuncture/line insertion site
Fluid overload
Allergic or anaphylactic reactions to plasma
Vasovagal episodes with fainting

Hypocalcemia
Thrombocytopenia
Tracheostomy 2
General explanation
Procedure to establish and maintain an open airway to provide adequate oxygenation
Percutaneous dilational tracheostomy is preferred:
Insertion of a cannula into the trachea
Insertion of dilators of gradually increasing size until the desired tracheostomy tube can
be accommodated
Indication
If, after 2 weeks, pulmonary function does not show significant improvement from baseline,
perform a tracheostomy 2
If pulmonary function rises above baseline, defer tracheostomy for an additional week,
allowing the patient to attempt to be weaned from the ventilator
Percutaneous tracheostomy is performed only in centers with adequate experience with the
technique 2
Complications 2
Tracheal stenosis
Hemorrhage
Infection
Fatal procedure-related necrotizing mediastinitis
Chyle fistula due to thoracic duct perforation
Permanent disfigurement owing to formation of a hypertrophic keloid tracheostomy scar
Special populations
Pregnant patients 26
Guillain-Barré syndrome is rare during pregnancy

Management parallels that of nonpregnant adults
Either plasma exchange or IV immunoglobulin is used for immunotherapy
Monitoring
Without close and attentive monitoring, rapid deterioration of the patient's condition may
be missed
Guillain-Barré syndrome typically progresses for 1 to 3 weeks after initial onset; hence,
monitoring is essential, especially for respiratory failure 3
Two-thirds of patients are unable to walk independently when maximum weakness is

reached 3
Respiratory insufficiency occurs in 25% of patients 3
Regularly monitor the following in patients with severe disease. Check every 2 to 4 hours if
the disease is progressing, and check every 6 to 12 hours if patient is in a steady state 3
Electrocardiography or cardiac monitor
Presence of cardiac arrhythmias
Serious arrhythmias or blood pressure swings occur in 20% of patients with

Guillain-Barré syndrome 2
Severe bradycardia causing asystole 2
Severe bradycardia may be preceded by wide blood pressure swings
Reduction in beat-to-beat variation in heart rate predicts dysautonomia requiring a

cardiac pacemaker 2
Blood pressure
Blood pressure fluctuations; wide swings exceeding 85 mm Hg systolic blood pressure

from day to day may predict development of bradycardia 2
Placement of invasive arterial blood pressure monitoring may be helpful
Pulse oximetry 3
Other measures of pulmonary function
Vital capacity; significant reduction to less than 15 mL/kg 3
Hypercarbia (PaCO₂ above 48 mm Hg) 3

Hypoxemia (PaO₂ below 56 mm Hg while the patient is breathing ambient air) 3
Swallowing
Check for significant impairment 2
Other symptoms of autonomic dysfunction
Ileus (perform daily abdominal auscultation) and pupil light unresponsiveness 1
During immunoglobulin therapy, check vital signs every 15 minutes during the first hour
and then check periodically thereafter 26
The Guillain-Barré syndrome disability score is widely used to provide an objective measure
of illness severity: 54
0: healthy state
1: minor symptoms, capable of running
2: able to walk 10 m or more without assistance but unable to run
3: able to walk meters across an open space with assistance
4: bedridden or chairbound
5: requiring assisted ventilation for at least part of the day
6: deceased
Complications and Prognosis
Complications
During the acute phase of disease, pneumonia, sepsis, pulmonary embolism, or
gastrointestinal bleeding develop in up to 60% of intubated patients with Guillain-Barré in
the ICU 2
Autonomic dysfunction manifests in two-thirds of patients as ileus, cardiac arrhythmias

possibly leading to asystole, atelectasis, and respiratory failure 1
Increased risk for deep vein thrombosis due to immobility
Severe fatigue has been reported in up to 80% of patients after the disease course 2
Women and patients over 50 years of age most frequently experience fatigue after illness
55
One-third of all patients still experience pain 1 year after onset 3
Among severely affected patients, 20% remain unable to walk 6 months after symptom onset
3
33% of patients infected with Campylobacter jejuni have prolonged severe disability 56
Patients may suffer from anxiety and/or depression due to persistent pain and disability
Prognosis
1 year after neuropathy onset, 65% of patients make a near-complete recovery and regain the
ability to perform manual work 3
Of the 35% who do not recover completely, about 8% will die in the acute stage, usually
from cardiac arrhythmias or pulmonary emboli 3
5% of patients with Guillain-Barré syndrome who live in developed countries die from
medical complications 3
Miller Fisher syndrome typically resolves without affecting the respiratory muscles and
has a benign course 42
2 or more episodes have been reported in 7% of patients 3
Mean interval between recurrences in these patients was 7 years 3
Prognostic factors indicating a worse outcome include:
Advanced age 4
Respiratory failure, associated with a reduction in vital capacity of greater than 20% 4
Guillain-Barré syndrome following Campylobacter jejuni infection 56
Compound muscle action potential amplitudes within 0% to 20% of the lower limit of
normal 16
Apply the Erasmus Guillain-Barré syndrome outcome score to patients after 2 weeks of
illness to predict those who will not be able to walk independently at 6 months (recovery is
largely complete at 6 months) 54
First requires deriving a Guillain-Barré syndrome disability score at 2 weeks after onset of
weakness
Presence of diarrhea and older age are predictive factors included in the Erasmus
Guillain-Barré syndrome outcome score
Total score ranges from 1 to 7
Age at onset
40 years and younger: 0 points
41 to 60 years: 0.5 point
Older than 60 years: 1 point
Diarrhea (within preceding 4 weeks)
Absent: 0 points
Present: 1 point
Guillain-Barré syndrome disability score (at 2 weeks into the illness)
0 or 1: 1 point
2: 2 points
3: 3 points
4: 4 points
5: 5 points
Scores of 3 points or less: 0.5% of these patients will be unable to walk without assistance
at 6 months
Score of 4 points: 7% of these patients will be unable to walk without assistance at 6

months
months
months
months
Screening and Prevention
Prevention
Due to the association with Campylobacter jejuni infection, efforts at reducing the
transmission of Campylobacter jejuni should reduce the incidence of Guillain-Barré
syndrome, particularly in less developed areas of the world. Reasonable prevention
measures include: 57
Improving sanitation
Cooking poultry products thoroughly
Disinfecting water supply
Avoiding raw milk consumption
Patients who have previously developed Guillain-Barré syndrome within 6 weeks of receipt
of influenza vaccine may defer influenza immunization. However, if provider thinks benefit
of protection (ie, reduced risk of influenza in patients with high risk of complications
caused by influenza) outweighs the small, theoretical risk of recurrence, provider may give
immunization 1
Recurrence of Guillain-Barré syndrome following influenza vaccination has not been

demonstrated. It is a theoretical risk in patients who had Guillain-Barré syndrome within
3 months or had developed the syndrome within 6 weeks of influenza vaccine
References
1 Willison HJ et al: Guillain-Barré syndrome. Lancet. 388(10045):717-27, 2016
View In Article | Cross Reference (https://pubmed.ncbi.nlm.nih.gov/26948435)
2 Hughes RA et al: Supportive care for patients with Guillain-Barré syndrome. Arch Neurol. 62(8):1194-8,
2005
3 Yuki N et al: Guillain-Barré syndrome. N Engl J Med. 366(24):2294-304, 2012

4 van Doorn PA et al: Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. Lancet
Neurol. 7(10):939-50, 2008
5 Dimachkie MM et al: Guillain-Barré syndrome and variants. Neurol Clin. 31(2):491-510, 2013
6 Walling AD et al: Guillain-Barré syndrome. Am Fam Physician. 87(3):191-7, 2013

7 Winer JB: Guillain Barré syndrome. Mol Pathol. 54(6):381-5, 2001

8 Koga M et al: Antecedent symptoms in Guillain-Barré syndrome: an important indicator for clinical
and serological subgroups. Acta Neurol Scand. 103(5):278-87, 2001
9 Fokke C et al: Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain. 137(Pt
1):33-43, 2014
10 Jones HR et al: Acute polyneuropathies. In: Darras BT et al, eds: Neuromuscular Disorders of Infancy,
Childhood, and Adolescence. 2nd ed. Academic Press; 2015:379-97
View In Article
11 Brannagan TH 3rd et al: HIV-associated Guillain-Barré syndrome. J Neurol Sci. 208(1-2):39-42, 2003
12 Esposito S et al: Guillain-Barré syndrome. Autoimmun Rev. 16(1):96-101, 2017

13 Katirji B: Disorders of peripheral nerves. In: Darof R et al, eds: Bradley's Neurology in Clinical Practice.
7th ed. Elsevier; 2016:1791-866.e12
View In Article
14 Kuwabara S et al: Axonal Guillain-Barré syndrome: concepts and controversies. Lancet Neurol.
12(12):1180-8, 2013
15 Tosun A et al: Acute motor-sensory axonal neuropathy with hyperreflexia in Guillain-Barré syndrome. J
Child Neurol. 30(5):637-40, 2015
16 Meena AK et al: Treatment guidelines for Guillain-Barré syndrome. Ann Indian Acad Neurol. 14(Suppl
1):S73-81, 2011
17 Sejvar JJ et al: Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis.
Neuroepidemiology. 36(2):123-33, 2011
18 Blum S et al: Genetics of Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating
polyradiculoneuropathy (CIDP): current knowledge and future directions. J Peripher Nerv Syst.
19(2):88-103, 2014
19 Geleijns K et al: The occurrence of Guillain-Barre syndrome within families. Neurology. 63(9):1747-50,
2004
20 Pritchard J et al: Guillain-Barré syndrome seen in users of isotretinoin. BMJ. 328(7455):1537, 2004
21 Shin IS et al: Guillain-Barré and Miller Fisher syndromes occurring with tumor necrosis factor alpha
antagonist therapy. Arthritis Rheum. 54(5):1429-34, 2006
22 Ropper AH et al: Guillain-Barré syndrome. In: Contemporary Neurology Series. FA Davis; 1991:57
View In Article
23 Aladawi M et al: Guillain Barre syndrome as a complication of COVID-19: a systematic review. Can J
Neurol Sci. 49(1):38-48, 2022
24 Abu-Rumeileh S et al: Guillain-Barré syndrome spectrum associated with COVID-19: an up-to-date

systematic review of 73 cases. J Neurol. 268(4):1133-70, 2021
25 Trujillo Gittermann LM et al: Relation between COVID-19 and Guillain-Barré syndrome in adults.
Systematic review. Neurologia. 35(9):646-54, 2020
26 Ansar V et al: Guillain-Barré syndrome. Prim Care. 42(2):189-93, 2015

27 Smith N et al: Investigation of suspected Guillain-Barre syndrome in childhood: what is the role for
gadolinium enhanced magnetic resonance imaging of the spine? J Paediatr Child Health. 50(10):E72-6,
2014
28 Byun WM et al: Guillain-Barré syndrome: MR imaging findings of the spine in eight patients.
Radiology. 208(1):137-41, 1998
29 Derksen A et al: Sural sparing pattern discriminates Guillain-Barré syndrome from its mimics. Muscle
Nerve. 50(5):780-4, 2014
30 Joffe AR: Lumbar puncture and brain herniation in acute bacterial meningitis: a review. J Intensive
Care Med. 22(4):194-207, 2007
31 Ruff RL et al: Complications of lumbar puncture followed by anticoagulation. Stroke. 12(6):879-81, 1981
32 McCombe PA et al: Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and

electrophysiological study of 92 cases. Brain. 110(Pt 6):1617-30, 1987
33 Sanchez JL: Clinical manifestations and treatment of Lyme disease. Clin Lab Med. 35(4):765-78, 2015
34 Mead P et al: Updated CDC recommendation for serologic diagnosis of Lyme disease. MMWR Morb
Mortal Wkly Rep. 68(32):703, 2019
35 Bissell DM et al: Acute hepatic porphyria. J Clin Transl Hepatol. 3(1):17-26, 2015
36 Simon NG et al: The neurologic manifestations of the acute porphyrias. J Clin Neurosci. 18(9):1147-53,
2011
37 Vrancken AF et al: Vasculitic neuropathy. Handb Clin Neurol. 115:463-83, 2013

38 Weber F et al: Hyperkalemic periodic paralysis. In: Pagon RA et al, eds: GeneReviews [internet].
University of Washington; 1993-2022
39 Jandhyala SN et al: Hypokalaemic periodic paralysis--a prospective study of the underlying etiologies. J
Clin Diagn Res. 9(9):OC17-9, 2015
40 Krishnan C et al: Transverse myelitis: pathogenesis, diagnosis and treatment. Front Biosci. 9:1483-99,
2004
41 Meriggioli MN et al: Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity.
Lancet Neurol. 8(5):475-90, 2009
42 Bushra JS: Miller Fisher syndrome: an uncommon acute neuropathy. J Emerg Med. 18(4):427-30, 2000
43 Ortiz-Salas P et al: Human immunoglobulin versus plasmapheresis in Guillain-Barre syndrome and

myasthenia gravis: a meta-analysis. J Clin Neuromuscul Dis. 18(1):1-11, 2016
44 Fujimura H: The Guillain-Barré syndrome. Handb Clin Neurol. 115:383-402, 2013

45 Hughes RA et al: Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the
Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 61(6):736-40, 2003
46 Abe T et al: The Japanese Society for Apheresis clinical practice guideline for therapeutic apheresis.
Ther Apher Dial. 25(6):728-876, 2021
47 Hughes RA et al: Corticosteroids for Guillain-Barré syndrome. Cochrane Database Syst Rev.
10:CD001446, 2016
48 Liu J et al: Pharmacological treatment for pain in Guillain-Barré syndrome. Cochrane Database Syst
Rev. 4:CD009950, 2015
49 Hiew FL et al: Hyponatraemia in Guillain-Barré syndrome revisited. Acta Neurol Scand. 133(4):295-301,
2016
50 Wang Y et al: Hyponatremia is a predictor for poor outcome in Guillain-Barré syndrome. Neurol Res.
37(4):347-51, 2015
51 Saifudheen K et al: Guillain-Barre syndrome and SIADH. Neurology. 76(8):701-4, 2011

52 van Doorn PA et al: IVIG treatment and prognosis in Guillain-Barré syndrome. J Clin Immunol. 30
Suppl 1:S74-8, 2010
53 JPAC: Therapeutic plasma exchange (TPE). In: Norfolk D, ed: Transfusion Handbook. 5th ed. TSO; 2013
View In Article | Cross Reference (http://www.transfusionguidelines.org.uk/transfusion-handbook/11-
therapeutic-apheresis/11-1-therapeutic-plasma-exchange-tpe)
54 van Koningsveld R et al: A clinical prognostic scoring system for Guillain-Barré syndrome. Lancet
Neurol. 6(7):589-94, 2007
55 Garssen MP et al: Residual fatigue is independent of antecedent events and disease severity in
Guillain-Barré syndrome. J Neurol. 253(9):1143-6, 2006
56 Hughes RA et al: Clinical and epidemiologic features of Guillain-Barré syndrome. J Infect Dis. 176
Suppl 2:S92-8, 1997
57 Nyati KK et al: Role of Campylobacter jejuni infection in the pathogenesis of Guillain-Barré syndrome:
an update. Biomed Res Int. 2013:852195, 2013
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CLINICAL OVERVIEW

Intubate or support patients who have respiratory failure with mechanical

Campylobacter jejuni is the most common precursory infection

Albuminocytologic dissociation, consisting of high levels of protein in the cerebrospinal

During the progressive phase, autonomic dysfunction manifests in two-thirds of patients,

Pneumonia, sepsis, pulmonary embolism, and gastrointestinal bleeding develop in up to

Campylobacter jejuni is the causative pathogen in 25% to 50% of adult patients 1

Acute inflammatory demyelinating polyneuropathy

Facial diplegia with paresthesias is a localized form of this subtype 3

Acute motor axonal neuropathy

Pharyngeal-cervical-brachial weakness—marked by acute weakness of the

There was a significant increase in this variant of Guillain-Barré syndrome during

This form is sometimes referred to as Chinese paralytic syndrome 4

Second most common subtype in Western countries (5%-10% of cases 6) 5

Acute motor-sensory axonal neuropathy 6

Rare subtype with more pronounced sensorial symptoms

Accounts for 5% to 10% of cases in the United States and Europe 4

Miller Fisher syndrome

Characterized by clinical symptoms relating to dysfunction of the third, fourth, and

Patients with diarrhea rarely show ophthalmoplegia or bulbar palsy

Characteristic symptom is progressive weakness of more than 2 limbs, with progression

Lower limbs as both proximal and distal weakness (56%) 5

Legs and arms simultaneously (32%) 5

Upper limbs (12%) 5

Typical course is acute progression of limb weakness, beginning as early as 12 hours

Respiratory muscle weakness tends to progress slowly and to be roughly correlated

Pain precedes weakness in one-third of adult patients with Guillain-Barré syndrome 3

Pain may manifest as dysesthesia or muscular, radicular, or arthralgic pain; pain is

Symptoms of autonomic dysfunction include the following:

Acute motor axonal neuropathy

Similar to acute inflammatory demyelinating polyneuropathy with these exceptions:

Abrupt onset of motor weakness and acute flaccid paralysis

Neuropathy is purely motor, although 10% of patients report distal paresthesias 12

Autonomic dysfunction is not present or is mild

Acute motor-sensory axonal neuropathy

Similar to acute inflammatory demyelinating polyneuropathy with more sensory

Has a more severe clinical course than acute inflammatory demyelinating

Miller Fisher syndrome

Distal paresthesia is closely associated with this variant

In children, weakness initially may be asymmetrical 10

There is occasional bilateral involvement of facial, oropharyngeal, and oculomotor

Hyporeflexia or areflexia is a hallmark of Guillain-Barré syndrome (acute inflammatory

Decreased tendon reflexes are common 1

Approximately 40% of children have loss of sensation (usually distal) 10

Facial paresis occurs in more than 50% of patients, usually bilateral 13

Extreme hypertension or hypotension; occurs in 20% of patients 3

Arrhythmias ranging from bradycardia to tachycardia

Bradycardia may be severe, possibly causing asystole 3

Abnormal pupillary response to light 1

Acute motor axonal neuropathy 14

Motor findings as per acute inflammatory demyelinating polyneuropathy except more

10% have normal or exaggerated reflexes throughout the illness

Acute motor-sensory axonal neuropathy

Presents as acute inflammatory demyelinating polyneuropathy with symmetrical

Rapidly progresses, with maximal deficit observed by day 7

Miller Fisher syndrome 1

Characterized by the triad of ophthalmoplegia, ataxia, and areflexia

Corneal reflex may be compromised 16

Causes and Risk Factors

Autoimmunity does not arise in 99% of people exposed to Guillain-Barré syndrome–