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Official reprint from UpToDate®

www.uptodate.com © 2023 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Guillain-Barré syndrome in children: Epidemiology,

clinical features, and diagnosis
Author: Monique M Ryan, FRACP
Section Editors: Douglas R Nordli, Jr, MD, Sheldon L Kaplan, MD, Jeremy M Shefner, MD, PhD
Deputy Editor: Richard P Goddeau, Jr, DO, FAHA

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Feb 2023. | This topic last updated: Sep 12, 2022.

INTRODUCTION

The acute immune-mediated polyneuropathies are classified under the eponym Guillain-Barré
syndrome (GBS) after some of the authors of early descriptions of the disease. GBS is one of the
most common causes of acute, acquired weakness and is often provoked by a preceding
infection. GBS may be complicated in some cases by respiratory failure and autonomic
dysfunction.

This epidemiology, clinical features, and diagnosis of GBS in children will be reviewed here.

Other aspects of GBS in children are discussed separately. (See "Guillain-Barré syndrome in
children: Treatment and prognosis".)

GBS in adults is presented elsewhere. (See "Guillain-Barré syndrome in adults: Pathogenesis,

clinical features, and diagnosis" and "Guillain-Barré syndrome in adults: Treatment and
prognosis".)

PATHOGENESIS

The pathogenesis of Guillain-Barré syndrome (GBS) is discussed here briefly and reviewed in
detail elsewhere. (See "Guillain-Barré syndrome in adults: Pathogenesis, clinical features, and
diagnosis".)

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One proposed mechanism for GBS is that an antecedent infection evokes an immune response,
which in turn cross-reacts with peripheral nerve components because of the sharing of cross-
reactive epitopes (molecular mimicry). The end result is an acute polyneuropathy. This immune
response can be directed toward the myelin or the axon of peripheral nerve. The main lesions
of GBS are acute inflammatory demyelinating polyradiculoneuropathy and, particularly in
patients with Campylobacter-associated disease, acute axonal degeneration. (See "Guillain-Barré
syndrome in adults: Pathogenesis, clinical features, and diagnosis" and "Guillain-Barré
syndrome in adults: Pathogenesis, clinical features, and diagnosis", section on 'Pathogenesis'.)

Antecedent infections are common with GBS and are thought to trigger the immune response
that leads to acute polyneuropathy. Approximately two-thirds of patients give a history of an
antecedent respiratory tract or gastrointestinal infection. Campylobacter infection is the most
commonly identified precipitant of GBS and can be demonstrated in as many as 30 percent of
cases. Other precipitants include cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae,
and influenza-like illnesses. GBS can also occur in association with HIV infection, predominantly
in those who are not profoundly immunocompromised. A small percentage of patients develop
GBS after another triggering event such as immunization, surgery, trauma, or bone-marrow
transplantation. (See "Guillain-Barré syndrome in adults: Pathogenesis, clinical features, and
diagnosis", section on 'Antecedent events'.)

While GBS has followed vaccinations, the small risk of GBS associated with influenza
vaccination, of approximately one to two excess cases of GBS per million people vaccinated, is
substantially less than the overall health risk posed by naturally occurring influenza.
Furthermore, the risk of GBS following influenza infection is several times greater than the risk
following influenza vaccination. (See "Guillain-Barré syndrome in adults: Pathogenesis, clinical
features, and diagnosis", section on 'Vaccinations'.)

The available evidence suggests that there is no increased risk of GBS associated with the H1N1
influenza vaccine in children [1-3].

EPIDEMIOLOGY

In the post-polio era, GBS is the most common cause of acute flaccid paralysis in healthy infants
and children [4,5]. GBS occurs worldwide with an annual incidence of 0.34 to 1.34 cases per
100,000 persons aged 18 years or less. [6-8]. While all age groups are affected, the incidence is
lower in children than in adults. The incidence increases by approximately 20 percent with every
10-year increase in age beyond the first decade of life. GBS occurs rarely in children younger

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than two years of age but can occur even in infants [9,10]. There is a case report of congenital
GBS in the neonate of an affected mother [11].

Males are affected approximately 1.5 times more often than females in all age groups.

CLINICAL FEATURES

GBS is a clinical syndrome with a number of variant forms. In patients with acute inflammatory
demyelinating polyradiculopathy (AIDP), the most common form of GBS, two-thirds develop the
neurologic symptoms two to four weeks after what initially appears to be a benign febrile
respiratory or gastrointestinal infection [12,13].

Neurologic symptoms — The classic presentation of GBS begins with paresthesia in the toes
and fingertips followed by lower extremity symmetric or modestly asymmetric weakness that
may ascend over hours to days to involve the arms and, in severe cases, the muscles of
respiration [14,15]. The predominant symptoms of GBS at presentation in children are pain and
gait difficulty [16]. In preschool-aged children, the most common symptoms are refusal to walk
and pain in the legs [17].

In a prospective series of 95 children with GBS, the most frequent initial symptoms were gait
unsteadiness, neuropathic pain, and inability to walk, occurring in 45, 34, and 24 percent of
cases, respectively [18]. By the peak of the illness, the frequency of symptoms was as follows:

● 79 percent had neuropathic pain.

● 60 percent could not walk.
● 51 percent had autonomic dysfunction.
● 46 percent had cranial nerve involvement.
● 24 percent could not use their arms.
● 13 percent required mechanical ventilation.

Cranial neuropathy most commonly affects the facial nerves, causing bilateral facial weakness
[18,19]. Pain typically involves the back and the legs [20].

Autonomic dysfunction occurs in approximately one-half of children with GBS and may include
the following [18,21]:

● A variety of cardiac dysrhythmias (asystole, bradycardia, persistent sinus tachycardia, and

atrial and ventricular tachyarrhythmias)
● Orthostatic hypotension
● Transient or persistent hypertension
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● Paralytic ileus
● Bladder dysfunction
● Abnormal sweating

Rare patients have central nervous system involvement [22].

Physical examination findings — Physical examination typically reveals symmetric weakness

with diminished or absent reflexes and gait abnormalities [14,15]. Sensory symptoms are
usually "positive" (eg, pain or paresthesia, reflecting nerve irritability) rather than "negative" (eg,
loss of sensation). Early symptoms may be atypical, rendering diagnosis difficult. Some cases
present with initial proximal weakness, or less common findings such as sphincter
disturbances, raising concerns about a possible spinal cord lesion.

Clinical course — More than 90 percent of patients reach the nadir of their function within two
to four weeks [23], followed by return of function occurring slowly over the course of weeks to
months.

The clinical course of GBS in children is shorter than in adults and recovery is usually more
complete [24-27]. In patients who did not require mechanical ventilation, the median time to
recovery of independent walking was 43 to 52 days in children compared with 85 days in adults
[28-30].

VARIANT FORMS OF GUILLAIN-BARRÉ SYNDROME

Historically, GBS was considered a single disorder. It is now known to be a heterogeneous

syndrome with several variant forms [6,31]. Acute inflammatory demyelinating
polyradiculopathy (AIDP) is the most common type. Axonal forms of GBS are also well
recognized. Each type of GBS has distinguishing clinical, pathophysiologic, and pathologic
features. The classic presentation of ascending paralysis is most common, but a number of
atypical variants present with local or regional involvement of particular muscle groups or
nerves [6,31,32]. Several have prominent cranial nerve involvement, including Miller Fisher
syndrome (MFS), Bickerstaff brainstem encephalitis, polyneuritis cranialis, and pharyngeal-
cervical-brachial weakness. Others include pure sensory neuropathy and acute
pandysautonomia.

Acute inflammatory demyelinating polyneuropathy — AIDP is the prototype of GBS and is

the most common form in North America, Europe, and most of the developed world, where it
accounts for about 85 to 90 percent of cases.

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Acute axonal neuropathies — These disorders are distinguished from AIDP by their more
severe course, involvement of motor or motor and sensory nerves, and an electrophysiologic
pattern suggesting axonal damage.

Acute motor axonal neuropathy — Acute motor axonal neuropathy (AMAN) is a pure motor
form of GBS. AMAN occurs mainly in northern China but is also a common form of GBS in other
locations, including Japan, Mexico, and South America [33-39]. It is more common in developing
nations, has a seasonal incidence, and is associated with a preceding Campylobacter jejuni
infection. In a series of 31 Japanese children with GBS classified by electrophysiologic criteria,
AIDP, AMAN, and unclassified GBS were seen in 11, 15, and 5 children (35, 48, and 16 percent,
respectively) [38].

The presenting clinical features and recovery are similar to those of AIDP [40]. However, more
patients have respiratory failure requiring assisted ventilation.

Acute motor-sensory axonal neuropathy — Acute motor-sensory axonal neuropathy

(AMSAN) resembles the motor axonal variant but has more sensory symptoms. The course
tends to be prolonged and severe [41,42]. The pathology is predominantly axonal lesions of
both motor and sensory nerve fibers. This form of GBS is uncommon in children.

GQ1b syndromes — Some forms of GBS characterized by eye movement abnormalities and
ataxia rather than limb weakness and numbness. These forms are associated with frequent
seropositivity to antibodies against the GQ1b ganglioside and include MFS, Bickerstaff
encephalitis, and pharyngeal-cervical-brachial weakness [43].

Miller Fisher syndrome — MFS is characterized by external ophthalmoplegia, ataxia, and

muscle weakness with areflexia [44,45]. Incomplete forms include acute ophthalmoplegia
without ataxia and acute ataxic neuropathy without ophthalmoplegia [6]. Cerebrospinal fluid
findings and electrophysiologic features are similar to those in acute inflammatory
demyelinating polyneuropathy. Brainstem auditory-evoked potentials demonstrate peripheral
and central conduction defects [27]. GQ1b antibodies are frequently found in patients with MFS
[46].

Bickerstaff encephalitis — Bickerstaff encephalitis is a brainstem encephalitis characterized

by encephalopathy and hyperreflexia, in combination with such features of MFS as
ophthalmoplegia and ataxia. It is not only clinically linked to MFS but is associated with anti-
GQ1b antibodies and can respond to intravenous immunoglobulin (IVIG) and plasma exchange
[47-49].

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Pharyngeal-cervical-brachial weakness — The pharyngeal-cervical-brachial variant of GBS is

characterized by acute weakness of the oropharyngeal, neck, and shoulder muscles, with
swallowing dysfunction [50-52]. Facial weakness may also be present. Strength and reflexes are
usually normal in the lower extremities. This form of GBS may overlap with MFS [51,53]. It is
thought to represent a localized form of axonal GBS [6,50,51]. Some patients with pharyngeal-
cervical-brachial weakness have immunoglobulin G (IgG) autoantibodies to GT1a, GQ1b, or (less
often) to GD1a.

Polyneuritis cranialis — Patients with polyneuritis cranialis develop acute bilateral multiple
cranial nerve involvement (eg, bilateral facial weakness, dysphagia, and dysphonia) with severe
peripheral sensory loss. Patients tend to be younger than those with other GBS subtypes. This
variant is associated with preceding cytomegalovirus infection [54].

Cerebrospinal fluid findings and electrophysiologic features of polyneuritis cranialis are similar
to those of AIDP. Magnetic resonance imaging (MRI) with gadolinium shows post-contrast
enhancement of the cranial nerve roots [55]. More children with this variant require ventilator
support than those with the more typical presentation of GBS [56]. However, most eventually
recover fully.

Other variants — There are a number of additional uncommon variants of GBS, including the
following:

● Acute pandysautonomia, symptoms of which include diarrhea, vomiting, dizziness,

abdominal pain, ileus, orthostatic hypotension, urinary retention, pupillary abnormalities,
an invariant heart rate, decreased sweating, salivation, and lacrimation [57]. The deep
tendon reflexes are absent or diminished and sensory symptoms may be present [58].

● Pure sensory GBS, with involvement of large sensory fibers leading to significant sensory
ataxia [59]. The deep tendon reflexes are absent and there may be minor motor
involvement. An association with antibodies to GD1b has been noted.

● Facial diplegia and distal limb paresthesia [53]. This is considered a variant of acute
inflammatory demyelinating polyneuropathy [6].

● Sixth nerve palsy and distal paresthesia [53].

● Bilateral lumbar radiculopathy [53].

● Paraparesis, with weakness restricted to the legs at presentation [50,60]. A minority

experiences some arm weakness over the course of the illness.

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DIAGNOSIS

The initial diagnosis of GBS is based upon the clinical presentation. The typical clinical features
of GBS are progressive, mostly symmetric or modestly asymmetric muscle weakness and
absent or depressed deep tendon reflexes (see 'Clinical features' above). The weakness can vary
from mild difficulty with walking to nearly complete paralysis of all extremity, facial, respiratory,
and bulbar muscles. However, some GBS variants present with local or regional involvement of
particular muscle groups or nerves, and several have prominent cranial nerve involvement; the
variable initial presentations can hinder early diagnosis. (See 'Variant forms of Guillain-Barré
syndrome' above.)

No single investigation can confirm or disprove the diagnosis of GBS, particularly early in its
course. In a child with a compatible clinical presentation, the diagnosis is contingent on
supporting evidence from the clinical examination, cerebrospinal fluid (CSF) analysis,
electrodiagnostic studies, magnetic resonance imaging (MRI), and/or ancillary investigations
(eg, serum immunoglobulin G [IgG] antibodies to GQ1b), with the exclusion of alternative
diagnoses by the same means.

Supportive features include the following:

● An elevated CSF protein (>45 mg/dL) with a normal CSF white blood cell count (ie,
albuminocytologic dissociation). (See 'Cerebrospinal fluid' below.)

● In the demyelinating forms of GBS, electrodiagnostic studies demonstrating abnormalities

including motor conduction block, slowing of motor and sensory nerve conduction,
temporal dispersion, and prolonged distal latencies. In the axonal forms of GBS, nerve
conduction studies showing decreased amplitude of motor (and possibly sensory)
responses, with normal conduction velocities. (See 'Electrodiagnostic studies' below.)

● Contrast enhancement of the spinal nerve roots, cauda equina, or cranial nerve roots on
MRI. (See 'Magnetic resonance imaging' below.)

● Detection of serum IgG antibodies to GQ1b, supporting the diagnosis of the GBS variants
Miller Fisher syndrome, Bickerstaff encephalitis, and pharyngeal-cervical-brachial
weakness. (See 'Antibodies' below.)

Cerebrospinal fluid — In patients with GBS, lumbar puncture often reveals an elevated CSF
protein with a normal CSF white blood cell count. This finding, known as albuminocytologic
dissociation, is present in 50 to 66 percent of patients with GBS in the first week after the onset

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of symptoms and ≥75 percent of patients in the third week [6,61,62]. The elevated protein may
be due to increased permeability of the blood-nerve barrier at the level of the proximal nerve
roots. A normal CSF protein is found in one-third to one-half of patients when tested earlier
than one week after symptom onset.

In a prospective series of 110 patients with GBS, initial CSF protein elevation varied from 45 to
200 mg/dL (0.45 to 2.0 g/L) in 73 percent of patients, but protein elevation as high as 1000
mg/dL (10 g/L) has been described [61]. As noted, the CSF cell count is typically normal (ie, <5
cells/mm3). However, a minority of patients with GBS has mildly elevated CSF cell counts. In the
same series, the CSF cell count was <5, 5 to 10, 11 to 30, and >30 cells/mm3 in 87, 9, 2, and 2
percent of patients, respectively [61].

Concurrent HIV infection or an alternative diagnosis (eg, Lyme disease, poliomyelitis,

enterovirus 71 infection, West Nile virus, or malignancy) should be considered in children with
acute flaccid paralysis who have a CSF cell count >50/mm3.

Electrodiagnostic studies — Electrodiagnostic studies are the most specific and sensitive tests
for diagnosis of GBS and establish the underlying pathophysiology as either demyelinating or
axonal. Performance of a detailed neurophysiologic study enables diagnosis of pediatric GBS in
as many as 90 percent of cases during the first week of symptoms [63]. Changes are virtually
universal by the second week of illness, by which time a definitive diagnosis can almost always
be made. Electrodiagnostic studies are uncomfortable and can be technically challenging in
small children; they should therefore be undertaken only by individuals with appropriate
pediatric expertise. (See "Overview of electromyography" and "Overview of nerve conduction
studies".)

Peripheral nerve demyelination often primarily affects proximal nerve roots and the terminal
segments of motor nerves and can be accompanied by conduction block [18,63,64]. In some
cases, this results in unrecordable motor responses. It may not be possible, on
neurophysiologic criteria, to distinguish this situation from extensive axonal degeneration. The
prognostic implications of severely reduced compound muscle action potential (CMAP)
amplitudes must therefore be interpreted with caution. When peripheral responses are
unrecordable, a search for more proximal responses (such as those from stimulation of the
phrenic or axillary nerves) may enable identification of a primarily demyelinating process.
Electromyographic evidence of acute denervation is always suggestive of severe axonal injury
and a worse prognosis [63-65].

Magnetic resonance imaging — Spinal MRI with administration of gadolinium frequently

shows enhancement of the spinal nerve roots and cauda equina during the first weeks after the

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onset of GBS in children [66-68]. The enhancement may be diffuse or predominantly involve the
ventral (anterior) nerve roots and less often the dorsal (posterior) roots. In some cases, nerve
root enhancement may be delayed and observed only on a repeat MRI. Enhancement of cranial
nerve roots may also be seen in some cases, reflecting more diffuse nerve involvement [68,69].

Evidence from uncontrolled retrospective studies suggests that the sensitivity of contrast-
enhanced spinal MRI for the diagnosis of childhood GBS is >90 percent [66-68]. Nevertheless,
spinal nerve root enhancement is a nonspecific finding that can be seen in a variety of disorders
including polyradiculopathy related to HIV or cytomegalovirus infection, chronic inflammatory
demyelinating polyneuropathy, arachnoiditis, sarcoidosis, carcinomatous or lymphomatous
meningitis, and certain metabolic diseases. Thus, the diagnosis of GBS cannot be made by MRI
alone.

Antibodies — Immune reactions directed against epitopes in Schwann cell surface membrane
or myelin can cause the acute demyelinating form of GBS, while immune reactions against
epitopes contained in the axonal membrane cause the acute axonal forms of GBS. Antibodies
against GQ1b, a ganglioside component of nerve, are present in the vast majority of patients
with Miller Fisher and similar syndromes. (See 'Miller Fisher syndrome' above and "Guillain-
Barré syndrome in adults: Pathogenesis, clinical features, and diagnosis", section on 'GQ1b
syndromes'.)

In clinical practice, commercially available testing for serum IgG antibodies to GQ1b is useful
for the diagnosis of Miller Fisher syndrome, having a sensitivity of 85 to 90 percent. Antibodies
to GQ1b may also be present in GBS with ophthalmoparesis, Bickerstaff encephalitis, and the
pharyngeal-cervical-brachial GBS variant but not in disorders other than GBS [46,70].

Currently, laboratory testing for antibodies to glycolipids other than GQ1b is not performed
routinely because of limited clinical utility.

DIFFERENTIAL DIAGNOSIS

Disorders of the central nervous system, peripheral nerve, neuromuscular junction, and muscle
may have features that initially resemble GBS ( table 1). However, consideration of the
neurologic examination, clinical course, cerebrospinal fluid profile, and electrodiagnostic
findings usually establish the diagnosis of GBS [23].

Chronic inflammatory demyelinating polyneuropathy — There is a temporal continuum

between acute inflammatory demyelinating polyneuropathy (AIDP), the demyelinating form of
GBS, and chronic inflammatory demyelinating polyneuropathy (CIDP).
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● AIDP is a monophasic subacute illness that reaches its nadir within three to four weeks.

● CIDP continues to progress or has relapses for longer than eight weeks. (See "Chronic
inflammatory demyelinating polyneuropathy: Etiology, clinical features, and diagnosis".)

● Subacute inflammatory demyelinating polyneuropathy is the term used by some authors

for disease that reaches its nadir between four and eight weeks.

This arbitrary temporal delineation of inflammatory demyelinating polyneuropathy can

occasionally be difficult to ascertain in practice. Only observation of the patient over time can
clarify whether the clinical course is that of AIDP or CIDP.

In addition to chronicity, other features may be useful to distinguish GBS (including AIDP) from
CIDP:

● While the onset of GBS is usually easily identified, the precise onset of CIDP is typically less
clear.

● Antecedent events are more frequent with GBS (where they occur in approximately 70
percent of cases) than with CIDP (where they are found in ≤30 percent of cases). (See
"Guillain-Barré syndrome in adults: Pathogenesis, clinical features, and diagnosis", section
on 'Antecedent events'.)

● Certain features are more consistent with an increased likelihood of CIDP in the first
weeks after onset of symptoms, including three or more episodes of clinical deterioration,
a mild disease course with retained ability to walk independently, and lack of cranial
neuropathies [71].

About 2 percent of patients initially diagnosed with AIDP will develop the chronic relapsing
weakness of CIDP. (See "Guillain-Barré syndrome in adults: Treatment and prognosis", section
on 'Approach to patients who relapse or worsen'.)

Other polyneuropathies — Acute polyneuropathies that may mimic GBS include those due to
acute severe vitamin B1 deficiency, acute arsenic poisoning, n-hexane (in glue sniffing
neuropathy), vasculitis, Lyme disease, tick paralysis, porphyria, sarcoidosis, leptomeningeal
disease, paraneoplastic disease, and critical illness.

The combination of data from the clinical setting, appropriate screening laboratory tests
(including thiamine level, rheumatologic testing, Lyme titer, spot urine for porphyria), as well as
electromyography with nerve conduction studies and cerebrospinal fluid analysis, are usually
sufficient to rule out these other, much less common, causes of polyneuropathy. A spot urine

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test for porphobilinogen in a sample obtained at the time of symptoms will identify the majority
of patients with acute porphyria. (See "Acute intermittent porphyria: Pathogenesis, clinical
features, and diagnosis".)

Peripheral nerve vasculitis is a potentially life-threatening illness that can be difficult to

diagnose. The pattern is that of a mononeuritis multiplex involving sensory and motor fibers in
the distribution of individual peripheral nerves. Although the pathology of the disease is
asymmetric, the clinical picture can mimic GBS with fairly symmetric ascending weakness when
the vasculitis is rapidly progressive with confluent nerve involvement. Vasculitic neuropathies
are rare in childhood. (See "Clinical manifestations and diagnosis of vasculitic neuropathies".)

Spinal cord disorders — Acute myelopathies due to spinal cord compression or acute
transverse myelitis can be confused with GBS, since reflexes can be depressed in the acute
stage of spinal cord disease. Early bowel and bladder dysfunction and a sensory level point to a
myelopathy. Imaging with spine MRI is usually helpful in diagnosing acute myelopathy by
demonstrating a focal spinal cord lesion.

Severe low back pain is common with GBS and frequently leads to imaging of the lumbar spine;
prominent contrast enhancement of the nerve roots on MRI may occur in GBS. (See 'Magnetic
resonance imaging' above.)

Neuromuscular junction disorders — Diseases of the neuromuscular junction including

botulism and myasthenia gravis can present with acute weakness, but sensory signs or
symptoms are lacking. Botulism is associated with large, unreactive pupils and constipation.
Electromyography with repetitive nerve stimulation and appropriate laboratory tests help clarify
the diagnosis. (See "Diagnosis of myasthenia gravis" and "Lambert-Eaton myasthenic
syndrome: Clinical features and diagnosis" and "Botulism".)

Muscle disorders — Acute polymyositis, critical illness myopathy, and critical illness neuropathy
can mimic GBS. The myopathy and neuropathy of critical illness present as an acute paralysis,
typically in patients receiving intensive care. High-dose intravenous glucocorticoids,
neuromuscular blocking drugs, sepsis, and multiorgan failure are thought to play an important
role, but the pathophysiology is not well understood. (See "Neuromuscular weakness related to
critical illness".)

SOCIETY GUIDELINE LINKS

Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Guillain-Barré
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syndrome".)

INFORMATION FOR PATIENTS

UpToDate offers two types of patient education materials, "The Basics" and "Beyond the
Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade
reading level, and they answer the four or five key questions a patient might have about a given
condition. These articles are best for patients who want a general overview and who prefer
short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more
sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading
level and are best for patients who want in-depth information and are comfortable with some
medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print
or e-mail these topics to your patients. (You can also locate patient education articles on a
variety of subjects by searching on "patient info" and the keyword(s) of interest.)

● Basics topics (see "Patient education: Guillain-Barré syndrome (The Basics)")

SUMMARY

● Pathogenesis – Guillain-Barré syndrome (GBS) is often triggered by an antecedent

infection that evokes an immune response, which in turn reacts with peripheral nerve
components because of the sharing of cross-reactive epitopes (molecular mimicry) to
cause an acute polyneuropathy. Campylobacter infection is the most commonly identified
precipitant of GBS. (See 'Pathogenesis' above.)

● Epidemiology – GBS is the most common cause of acute flaccid paralysis in healthy
infants and children. The estimated annual incidence in children is 0.34 to 1.34 cases per
100,000. The incidence increases by approximately 20 percent with every 10-year increase
in age beyond the first decade of life. Males are affected approximately 1.5 times more
often than females. (See 'Epidemiology' above.)

● Clinical features – The classic presentation of GBS begins with paresthesia in the toes and
fingertips followed by lower extremity fairly symmetric weakness that may ascend over
hours to days to involve the arms and, in severe cases, the muscles of respiration. Cranial
nerve and autonomic dysfunction may also occur. (See 'Neurologic symptoms' above.)

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Most patients reach their clinical nadir within two to four weeks, with subsequent return
of function over the course of weeks to months. (See 'Clinical course' above.)

● Variant forms – GBS is a heterogeneous syndrome with several variant forms (see
'Variant forms of Guillain-Barré syndrome' above):

• Acute inflammatory demyelinating polyneuropathy is the most common form of GBS.

It is due to demyelinating injury and typically presents with ascending limb paralysis.
(See 'Acute inflammatory demyelinating polyneuropathy' above.)

• Acute motor or motor-sensory neuropathies represent typically severe forms of GBS

due to axonal damage. (See 'Acute axonal neuropathies' above.)

• The Miller Fisher syndrome, Bickerstaff brainstem encephalitis, and pharyngeal-

cervical-brachial weakness are forms of GBS characterized by eye movement
abnormalities and ataxia and are associated with frequent seropositivity to antibodies
against the GQ1b ganglioside. (See 'GQ1b syndromes' above.)

• Other rare syndromes include presentations of primarily cranial nerve, sensory, or

autonomic dysfunction. (See 'Polyneuritis cranialis' above and 'Other variants' above.)

● Diagnosis – The clinical diagnosis of GBS is made in patients with typical features of
progressive, mostly symmetric muscle weakness and absent or depressed deep tendon
reflexes. The clinical diagnosis is supported by findings on diagnostic testing that suggest
GBS and help exclude alternative diagnoses. Testing may include the following (see
'Diagnosis' above):

• Cerebrospinal fluid (CSF) may show an albuminocytologic dissociation, characterized by

an elevated CSF protein (>45 mg/dL) with a normal CSF white blood cell count. (See
'Cerebrospinal fluid' above.)

• Electrodiagnostic studies may demonstrate abnormalities indicating demyelination

including motor conduction block, slowing of motor and sensory nerve conduction,
temporal dispersion, and prolonged distal latencies. Decreased amplitude of motor
(and possibly sensory) responses may be seen in axonal forms of GBS. (See
'Electrodiagnostic studies' above.)

• Spinal MRI with administration of gadolinium frequently shows enhancement of the

spinal nerve roots and cauda equina. (See 'Magnetic resonance imaging' above.)

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• Antibodies against GQ1b, a ganglioside component of nerve, are present in many

patients with Miller Fisher and similar syndromes. (See 'Antibodies' above.)

● Differential diagnosis – Disorders of the central nervous system, peripheral nerve,

neuromuscular junction, and muscle may have features that initially resemble GBS
( table 1). However, the presentation, neurologic examination, clinical course,
cerebrospinal fluid profile, and electrodiagnostic findings usually establish the diagnosis
of GBS. (See 'Differential diagnosis' above.)

ACKNOWLEDGMENT

The UpToDate editorial staff acknowledges Robert Cruse, DO, who contributed to earlier
versions of this topic review.

Use of UpToDate is subject to the Terms of Use.

REFERENCES

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Topic 6235 Version 23.0

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GRAPHICS

Differential diagnosis of Guillain-Barré syndrome

Differential diagnosis

Cerebral

Acute disseminated encephalomyelitis (ADEM)

Bilateral strokes

Psychogenic symptoms

Cerebellar

Acute cerebellar ataxia syndrome

Posterior fossa structural lesion

Spinal

Anterior spinal artery syndrome

Compressive myelopathy

Neuromyelitis optica

Poliomyelitis

Transverse myelitis

Other infectious causes of acute myelitis (eg, West Nile virus, coxsackieviruses, echoviruses)

Peripheral nervous system

Acute onset chronic inflammatory demyelinating neuropathy (CIDP)

Critical illness polyneuropathy

Cytomegalovirus-related radiculitis

Diphtheria

HIV-related radiculitis

Leptomeningeal malignancy

Lyme disease

Metabolic and electrolyte disorders (eg, hypoglycemia, hypophosphatemia)

Porphyria

Thiamine deficiency (beriberi)

Tick paralysis

Toxic neuropathy

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Vasculitis

Neuromuscular junction

Botulism

Myasthenia gravis

Neuromuscular blocking agents

Muscle disease

Acute inflammatory myopathies (eg, dermatomyositis, polymyositis)

Acute viral myositis

Acute rhabdomyolysis

Critical illness myopathy

Metabolic myopathies (eg, hypokalemic, hyperkalemic)

Mitochondrial myopathies

Periodic paralysis

Data from:
1. Evans OB. Guillain-Barré syndrome in children. Pediatr Rev 1986; 8:69.
2. Jones HR. Childhood Guillain-Barré syndrome: clinical presentation, diagnosis, and therapy. J Child Neurol 1996; 11:4.
3. Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med 2012; 366:2294.
4. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet 2016; 388:717.

Graphic 80040 Version 7.0

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Contributor Disclosures
Monique M Ryan, FRACP Grant/Research/Clinical Trial Support: Biogen, Inc [Spinal muscular atrophy];
Novartis [Spinal muscular atrophy]. Consultant/Advisory Boards: AveXis [Spinal muscular atrophy]; Biogen,
Inc [Spinal muscular atrophy]; BioMarin [CLN2 disease]. Speaker's Bureau: Biogen, Inc [Spinal muscular
atrophy]; Novartis [Spinal muscular atrophy]; Roche [Spinal muscular atrophy]. All of the relevant financial
relationships listed have been mitigated. Douglas R Nordli, Jr, MD No relevant financial relationship(s)
with ineligible companies to disclose. Sheldon L Kaplan, MD Grant/Research/Clinical Trial Support:
MeMed Diagnostics [Bacterial and viral infections]; Merck [Staphylococcus aureus]; Pfizer [Streptococcus
pneumoniae]. Consultant/Advisory Boards: MeMed Advisory Board [Diagnostics bacterial and viral
infections]. Other Financial Interest: Elsevier [Pediatric infectious diseases]; Pfizer [PCV13]. All of the
relevant financial relationships listed have been mitigated. Jeremy M Shefner, MD,
PhD Grant/Research/Clinical Trial Support: AB Sciences [ALS]; Acorda Therapeutics [ALS]; Alector [ALS];
Amylyx [ALS]; Biogen [ALS]; Cytokinetics Incorporated [ALS]; Ionis [ALS]; Mitsubishi Tanabe Pharma
America [ALS]; PTC [ALS]; QurAlis [ALS]; Sanofi [ALS]; Wave Life Sciences [ALS]. Consultant/Advisory Boards:
AcuraStem [ALS]; Amylyx [ALS]; Apellis [ALS]; Cytokinetics [ALS]; Denali [ALS]; GSK [ALS]; Mitsubishi Tanabe
Pharma America [ALS]; Neurosense [ALS]; Novartis [ALS]; Orthogonal [ALS]; Pinteon [ALS]; Revalesio [ALS];
RRD [ALS]; Swanbio [ALS]. All of the relevant financial relationships listed have been mitigated. Richard P
Goddeau, Jr, DO, FAHA No relevant financial relationship(s) with ineligible companies to disclose.

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
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