Guillain-Barré Syndrome

Guillain-Barré syndrome
Prof. Dr. Saad S Al Ani

Senior Pediatric Consultant
Head of Pediatric Department
Khorfakkan Hospital
Sharjah , UAE
saadsalani@yahoo.com
Guillain-Barré syndrome (GBS)
A collection of clinical syndromes that

manifests as an acute inflammatory
polyradiculoneuropathy with resultant
weakness and diminished reflexes.
https://emedicine.medscape.com
Pediatric Guillain-Barré syndrome

19/08/2018 2
Prof. Dr .Saad S Al Ani
Overview
The classic presentation is
characterized by an acute
monophasic, non-febrile, post-
infectious illness manifesting as
ascending weakness and areflexia

19/08/2018 3
Overview (cont.)
• Sensory, autonomic, and brainstem

abnormalities may also be seen.
• With the eradication of poliomyelitis,
GBS is the most common cause of
acute motor paralysis in children.

19/08/2018 4
Pathogenesis
• The pathogenesis of GBS remains
unclear.
Mayo Clinic

19/08/2018 5
Pathogenesis (cont.)
• Increasing data indicate that it is an

autoimmune disease, often triggered
by a preceding viral or bacterial
infection with organisms such as:
 Campylobacter jejuni
 Cytomegalovirus
 Epstein-Barr virus
 Mycoplasma pneumoniae.
Kuwabara S. Guillain-Barré syndrome: epidemiology, pathophysiology
and management. Drugs. 2004. 64(6):597-610

19/08/2018 6
Pathogenesis (cont.)
• Vaccination against the:

 Flu
 Rabies
 Meningitis
are documented precipitating factors
Kuwabara S. Guillain-Barré syndrome: epidemiology, pathophysiology
and management. Drugs. 2004. 64(6):597-610

19/08/2018 7
Pathophysiology
Two pathophysiological forms have been
described:
 Demyelinating form of GBS
 Axonal forms of GBS

19/08/2018 8
Pathophysiology (Cont.)
Demyelinating form of GBS :

Segmental demyelination of peripheral
nerves is due to immune mediated
involving both humoral and cell-
mediated immune mechanisms

19/08/2018 9
19/08/2018 10
Axonal forms of GBS

axonal degeneration may occur
without demyelination or
inflammation.

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Science Direct

19/08/2018 12
of patients have a history of an

antecedent gastrointestinal or
respiratory tract infection

19/08/2018 13
The mechanism of disease possibly

involves an abnormal T-cell response
precipitated by an infection which
activate CD4+ helper-inducer T cells
Kimoto K, Koga M, Odaka M, Hirata K, Takahashi M, Li J, et al. Relationship of
bacterial strains to clinical syndromes of Campylobacter-associated
neuropathies. Neurology. 2006 Nov 28. 67(10):1837-43

19/08/2018 14
Epidemiology
The annual incidence of GBS range
from 0.5-1.5 cases per 100,000
population in individuals younger
than 18 years
Landaverde JM, Danovaro-Holliday MC, Trumbo SP, Pacis-Tirso CL,

Ruiz-Matus C. Guillain-Barré syndrome in children aged J Infect
Dis</i>. 2010 Mar. 201(5):746-50.

19/08/2018 15
Epidemiology (Cont.)
No evidence exists for any racial

predilection
Males appear to be at greater risk
for GBS than females
Korinthenberg R, Schessl J, Kirschner J. Clinical presentation and

course of childhood Guillain-Barré syndrome: a prospective
multicentre study. Neuropediatrics. 2007 Feb. 38(1):10-7.

19/08/2018 16
Subtypes of GBS
The clinical spectrum of GBS, which includes
individual variation and variable severity of
presentation, comprises the following:
1. Acute inflammatory demyelinating
polyradiculoneuropathy (AIDP)
2. Acute motor axonal neuropathy (AMAN)
3. Acute motor and sensory axonal neuropathy
(AMSAN)
4. Miller-Fisher syndrome (MFS)
5. Polyneuritis cranialis
Kieseier BC, Kiefer R, Gold R, Hemmer B, Willison HJ, Hartung HP. Advances in understanding and
treatment of immune-mediated disorders of the peripheral nervous system. Muscle Nerve. 2004
Aug. 30(2):131-56

19/08/2018 17
1.Acute inflammatory demyelinating
polyradiculoneuropathy (AIDP)
• Accounts for 80-90% of GBS cases
( Europe and North America)
• Characterized by an immune-mediated
attack on myelin with infiltration of
lymphocytes and macrophages with
segmental stripping of myelin.

19/08/2018 18
http://www.emgtest.com/wp-content/uploads/2012/11/GUILIAN-BARRE-SX.jpg

19/08/2018 19
1.Acute inflammatory demyelinating
polyradiculoneuropathy (AIDP) (Cont.)
• Motor and sensory fibres are usually
affected simultaneously, producing
corresponding deficits.
• Electrophysiology shows:
1. Slow nerve conduction velocity
2. Prolonged F waves.

19/08/2018 20
2.Acute motor axonal neuropathy
(AMAN)
• Most commonly seen in China and
Japan (50-60% of cases), as apposed to
Western countries (10-20% of cases).
• In this form, axonal degeneration occurs
by immune attack within 1-2 weeks
after infection.

19/08/2018 21
(AMAN) (cont.)
• Specific antibodies to axonal membranes
of motor fibres attack the nodes of
Ranvier.
• This, in turn, activates complement and
intrusion of macrophages into
periaxonal space, resulting in
destruction of axons.

19/08/2018 22
http://www.emgtest.com/wp-content/uploads/2012/11/GUILIAN-BARRE-SX.jpg

19/08/2018 23
(AMAN) (Cont.)
• C jejuni is the most common preceding
infection, and antiganglioside antibodies
are usually found in this type.
• Electrophysiology shows:
1. Reduction in muscle action potentials
with relatively preserved motor nerve
conduction velocity
2. Normal sensory nerve action potentials
Schwerer B. Antibodies against gangliosides: a link between preceding
and F waves
infection and immunopathogenesis of Guillain-Barré syndrome. Microbes
Infect. 2002 Mar. 4(3):373-84
19/08/2018 24
3.Acute motor and sensory axonal
neuropathy (AMSAN)
• This type is rare and resembles

AMAN except sensory nerves are
also affected.
• This type is associated with a severe
course and poor prognosis.
Schwerer B. Antibodies against gangliosides: a link between preceding infection and
immunopathogenesis of Guillain-Barré syndrome. Microbes Infect. 2002 Mar. 4(3):373-84

19/08/2018 25
4.Miller-Fisher syndrome
(MFS)
• The involvement of CNs is very
distinct in this form of GBS.
• Ocular motor nerves (oculomotor,
trochlear, and abducens) are
affected and produce a triad of
ophthalmoplegia, ataxia, and
areflexia.
19/08/2018 26
4.Miller-Fisher syndrome
(MFS) (Cont.)
• Electrophysiology is normal.
• The characteristic autoantibodies are
against gangliosides GQ1b and
GT1a.
• GQ1b plays a key role in the
pathogenesis of MFS.
19/08/2018 27
Science Direct

19/08/2018 28
Polyneuritis cranialis
• This is an acute onset of Multiple CN
palsies (usually bilateral CN VII with
sparing of CNs I and II)
o Elevated cerebrospinal fluid protein
o Slowed nerve conduction velocity
o Uncomplicated recovery.

19/08/2018 29
Physical Examination
• An ascending motor weakness is noted
along with areflexia in the classic form.
• Areflexia is a hallmark of GBS.
• Occasionally, some of the more
proximal reflexes still may be elicited
during the early phase of the disease.

19/08/2018 30
Physical Examination (Cont.)
• Of clinical value is documenting reflexes

in serial exams.
• Progression from normoreflexia /
hyporeflexia to areflexia is consistent with
acute features of GBS.

19/08/2018 31
• Occasionally:
o Autonomic instability (26%)
o Ataxia (23%)
o Dysesthesias (20%)
o Cranial nerve findings (35-50%),
predominantly facial palsy (Children>adult)
are noted.
Inaloo S, Katibeh P. Guillain-barre syndrome presenting with bilateral
facial nerve palsy. Iran J Child Neurol. 2014 Winter. 8(1):70-2
19/08/2018 32
• Leg weakness (i.e., foot drop) is

usually noticed first and weakness
eventually involves the calves and
thighs.
• Later, respiratory muscles and
upper extremities show involvement.

19/08/2018 33
• Some children may become non-ambulatory.

• Weakness also may involve the respiratory
muscles, and some children need respiratory
support during the course of the disease.
• Mechanical ventilation is used until
respiratory muscle function returns.

19/08/2018 34
• The autonomic neuropathy involves both

the sympathetic and parasympathetic
systems; manifestations include:
 Orthostatic hypotension
 Hypertension
 Pupillary dysfunction
 Sweating abnormalities
 Sinus tachycardia
19/08/2018 35
Guillain-Barré syndrome
time course
https://www.thelancet.com/action/show
19/08/2018 36
Diagnosis
The diagnosis of GBS is typically based on

the presence of :
o Progressive ascending weakness
o Areflexia

19/08/2018 37
Diagnosis (Cont.)
• Findings on :
 Lumbar puncture
 Electrodiagnostic studies
 MRI (occasionally)
Can give support for the diagnosis.
• Abnormalities on these studies do not develop
until days to weeks after onset of symptoms.

19/08/2018 38
Diagnosis (Cont.)
• Findings on :
 Lumbar puncture
 Electrodiagnostic studies
 MRI (occasionally)
Can give support for the diagnosis.
• Abnormalities on these studies do not develop
until days to weeks after onset of symptoms.

19/08/2018 39
Lumbar Puncture
Typically, the LP findings are suggestive of
demyelination (i.e., increased protein >45
mg/dL within 3 weeks of onset) without
evidence of active infection (lack of CSF
pleocytosis),

19/08/2018 40
Lumbar Puncture (cont.)
• The CSF findings may be normal within the

first 48 hours of symptoms
• Occasionally the protein may not rise for a
week.
• Usually by 10 days of symptoms, elevated CSF
protein findings will be most prominent.

19/08/2018 41
Lumbar Puncture (Cont.)
• Most patients have fewer than 10 leukocytes

per milliliter, but occasionally a mild
elevation (i.e., 10-50 cells/mL) is seen.
• Greater than 50 mononuclear cells/mL of CSF
makes the diagnosis of GBS doubtful.

19/08/2018 42
Electrodiagnostic Studies
Within the first week of the onset of symptoms,
electrodiagnostic studies in at least two limbs reveal
the following:
• A dispersed, impersistent, prolonged, or absent F
response (88%)
• Increased distal latencies (75%)
• Conduction block (58%) or temporal dispersion of
compound muscle action potential (CMAP)
• Reduced conduction velocity (50%) of motor and
sensory nerves

19/08/2018 43
Electrodiagnostic Studies (cont.)
Criteria for axonal forms include:

o Lack of neurophysiologic evidence of
demyelination
oLoss of amplitude of CMAP or sensory
nerve action potentials to at least less
than 80% of lower limit of normal
values for age

19/08/2018 44
Serum Anti-Ganglioside
Antibodies
• In adults with GBS, serum ganglioside
antibodies directed against GM1, GM1b,
GD1a, and GalNAc-GDIa have been
associated with Campylobacter
jejuni infection, acute motor axonal
neuropathy, a more severe course, and more
residual neurologic deficits.
• The value of these studies as a prognostic
marker in children is still under evaluation.
19/08/2018 45
19/08/2018 46
Diagnosis (Cont.)
Nearly 2 weeks after presentation of symptoms,

lumbosacral MRI can show enhancement of
the nerve roots with gadolinium.
 This imaging study has been described to be
83% sensitive for acute GBS, with nerve root
enhancement present in 95% of typical cases
Gorson KC, Ropper AH, Muriello MA, Blair R. Prospective evaluation of MRI lumbosacral nerve root
enhancement in acute Guillain-Barré syndrome. Neurology. 1996 Sep. 47(3):813-7

19/08/2018 47
Spinal cord lesions may be considered in the differential
diagnosis:
• Transverse myelitis • Vascular malformations
• Epidural abscess • Cord infarctions
• Tumors • Cord compression
• Enteroviral infections of • Lumbosacral disk
the anterior horn cells syndromes
• Poliomyelitis • Trauma
• Hopkins syndrome

19/08/2018 48
Peripheral neuropathies from the following may
produce a GBS-like picture:
• Vincristine • Glue sniffing
• Heavy metals poisoning • Organophosphate pesticides
• HIV infection • Diphtheria
• Lyme disease • Inborn errors of metabolism
• Leigh disease • Tangier disease
• Porphyria

19/08/2018 49
Treatment
• In pediatrics, the most effective form of

therapy is generally considered to
be intravenous immunoglobulin (IVIG)
• Plasmapheresis may also be used

19/08/2018 50
Prognosis
• In general, the outcome of GBS is
more favourable in children than in
adults
• the recovery period is long, often
weeks to months

19/08/2018 51
Prognosis (cont.)
• Rarely, it can be fatal in 5-10% of

patients with respiratory failure and
cardiac arrhythmia
• Recurrence of GBS occurs in
approximately 5% of cases

19/08/2018 52
Prognosis
• Overall mortality rate in childhood
GBS is estimated to be less than 5%
• Deaths are usually caused by
respiratory failure, often in
association with :
 Cardiac arrhythmias
 Dysautonomia
19/08/2018 53
Complications of GBS
• The most common serious complications

are:
1. Weakness of the respiratory muscles
2. Autonomic instability
Ilyas M, Tolaymat A. Minimal change nephrotic syndrome with

Guillain-Barré syndrome. Pediatr Nephrol. 2004 Jan. 19(1):105-6

19/08/2018 54
Complications of GBS (Cont.)
Other important potential complications

include:
• Pneumonia • Ileus
• Adult respiratory distress • constipation
syndrome
• Septicemia • gastritis
• Pressure sores • dysesthesias
• Pulmonary embolus • Nephropathy
Ilyas M, Tolaymat A. Minimal change nephrotic syndrome with
Guillain-Barré syndrome. Pediatr Nephrol. 2004 Jan. 19(1):105-6

19/08/2018 55
Clinical Summary
Features that would put the diagnosis in doubt
include:
(1) Marked persistent weakness
(2) Bowel and bladder dysfunction at onset
(3) Persistent bladder or bowel dysfunction
(4) Mononuclear leukocytosis in the
cerebrospinal fluid (>50 cells/µL)
(5) A sharp sensory level
19/08/2018 56
Clinical Summary (Cont.)
Features required for diagnosis are:

(1) Progressive weakness of more than one
extremity
(2) Hyporeflexia or areflexia
(3) Elevated cerebrospinal fluid protein (>45
mg/dL) after 1 week following onset of
symptoms
(4) Slow conduction velocity or prolonged F
wave on electrophysiology testing.
19/08/2018 57
Clinical Summary (Cont.)
Features that rule out the diagnosis include:

(1) A current history of hexacarbon abuse
(2) Abnormal porphyria metabolism
(3) Recent diphtheria infection
(4) Evidence of polio, botulism, toxic
neuropathy, tic paralysis, or
organophosphate poisoning.
19/08/2018 58
References
• Kuwabara S. Guillain-Barré syndrome: epidemiology, pathophysiology and management. Drugs. 2004.
64(6):597-610
• Landaverde JM, Danovaro-Holliday MC, Trumbo SP, Pacis-Tirso CL, Ruiz- Matus C. Guillain-Barré
syndrome in children aged J Infect Dis</i>. 2010 Mar. 201(5):746-50
• Kimoto K, Koga M, Odaka M, Hirata K, Takahashi M, Li J, et al. Relationship of bacterial strains to
clinical syndromes of Campylobacter-associated neuropathies. Neurology. 2006 Nov 28.
67(10):1837-43
• Korinthenberg R, Schessl J, Kirschner J. Clinical presentation and course of childhood Guillain-
Barré syndrome: a prospective multicentre study. Neuropediatrics. 2007 Feb. 38(1):10-7.
• Kieseier BC, Kiefer R, Gold R, Hemmer B, Willison HJ, Hartung HP. Advances in understanding
and treatment of immune-mediated disorders of the peripheral nervous system. Muscle Nerve.
2004 Aug. 30(2):131-56
• Schwerer B. Antibodies against gangliosides: a link between preceding infection and
immunopathogenesis of Guillain-Barré syndrome. Microbes Infect. 2002 Mar. 4(3):373-84
• Inaloo S, Katibeh P. Guillain-Barré syndrome presenting with bilateral facial nerve palsy. Iran J
Child Neurol. 2014 Winter. 8(1):70-2
• Ilyas M, Tolaymat A. Minimal change nephrotic syndrome with Guillain-Barré syndrome. Pediatr
.
Nephrol. 2004 Jan. 19(1):105-6
• Gorson KC, Ropper AH, Muriello MA, Blair R. Prospective evaluation of MRI lumbosacral nerve
root enhancement in acute Guillain-Barré syndrome. Neurology. 1996 Sep. 47(3):813-7

19/08/2018 59
19/08/2018 60

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Guillain-Barré Syndrome

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Guillain-Barré syndrome

Prof. Dr. Saad S Al Ani

A collection of clinical syndromes that

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

• Sensory, autonomic, and brainstem

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

• Increasing data indicate that it is an

Pediatric Guillain-Barré syndrome

• Vaccination against the:

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Demyelinating form of GBS :

Pediatric Guillain-Barré syndrome

Axonal forms of GBS

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

of patients have a history of an

Pediatric Guillain-Barré syndrome

The mechanism of disease possibly

Pediatric Guillain-Barré syndrome

Landaverde JM, Danovaro-Holliday MC, Trumbo SP, Pacis-Tirso CL,

Pediatric Guillain-Barré syndrome

No evidence exists for any racial

Korinthenberg R, Schessl J, Kirschner J. Clinical presentation and

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

• This type is rare and resembles

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

• Of clinical value is documenting reflexes

Pediatric Guillain-Barré syndrome

• Leg weakness (i.e., foot drop) is

Pediatric Guillain-Barré syndrome

• Some children may become non-ambulatory.

Pediatric Guillain-Barré syndrome

• The autonomic neuropathy involves both

The diagnosis of GBS is typically based on

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

• The CSF findings may be normal within the

Pediatric Guillain-Barré syndrome

• Most patients have fewer than 10 leukocytes

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Criteria for axonal forms include:

Pediatric Guillain-Barré syndrome

Nearly 2 weeks after presentation of symptoms,

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

Pediatric Guillain-Barré syndrome

• In pediatrics, the most effective form of