Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy that causes weakness and diminished reflexes. It is typically characterized by an acute monophasic illness manifesting as ascending weakness and areflexia following a gastrointestinal or respiratory infection. The classic presentation is acute inflammatory demyelinating polyradiculoneuropathy but it can also present as acute axonal forms that damage nerve axons. Diagnosis is based on progressive ascending weakness, areflexia, and supportive findings on lumbar puncture, electrodiagnostic studies, and occasionally MRI.
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Guillain - Barré syndrome
1. Guillain-Barré syndrome
Prof. Dr. Saad S Al Ani
Senior Pediatric Consultant
Head of Pediatric Department
Khorfakkan Hospital
Sharjah , UAE
saadsalani@yahoo.com
2. A collection of clinical syndromes that
manifests as an acute inflammatory
polyradiculoneuropathy with resultant
weakness and diminished reflexes.
Guillain-Barré syndrome (GBS)
19/08/2018
Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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https://emedicine.medscape.com
3. Overview
The classic presentation is
characterized by an acute
monophasic, non-febrile, post-
infectious illness manifesting as
ascending weakness and areflexia
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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4. 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.
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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5. Pathogenesis
• The pathogenesis of GBS remains
unclear.
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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Mayo Clinic
6. 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.
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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Kuwabara S. Guillain-Barré syndrome: epidemiology, pathophysiology
and management. Drugs. 2004. 64(6):597-610
7. Pathogenesis (cont.)
• Vaccination against the:
Flu
Rabies
Meningitis
are documented precipitating factors
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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Kuwabara S. Guillain-Barré syndrome: epidemiology, pathophysiology
and management. Drugs. 2004. 64(6):597-610
8. Pathophysiology
Two pathophysiological forms have been
described:
Demyelinating form of GBS
Axonal forms of GBS
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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9. Pathophysiology (Cont.)
Demyelinating form of GBS :
Segmental demyelination of peripheral
nerves is due to immune mediated
involving both humoral and cell-
mediated immune mechanisms
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Pediatric Guillain-Barré syndrome
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11. Pathophysiology (Cont.)
Axonal forms of GBS
axonal degeneration may occur
without demyelination or
inflammation.
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14. Pathophysiology (Cont.)
The mechanism of disease possibly
involves an abnormal T-cell response
precipitated by an infection which
activate CD4+ helper-inducer T cells
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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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
15. Epidemiology
The annual incidence of GBS range
from 0.5-1.5 cases per 100,000
population in individuals younger
than 18 years
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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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.
16. Epidemiology (Cont.)
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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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.
No evidence exists for any racial
predilection
Males appear to be at greater risk
for GBS than females
17. 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
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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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
18. 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.
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20. 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.
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Pediatric Guillain-Barré syndrome
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21. 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.
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Pediatric Guillain-Barré syndrome
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22. 2.Acute motor axonal neuropathy
(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.
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Pediatric Guillain-Barré syndrome
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24. 2.Acute motor axonal neuropathy
(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
and F waves
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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Schwerer B. Antibodies against gangliosides: a link between preceding
infection and immunopathogenesis of Guillain-Barré syndrome. Microbes
Infect. 2002 Mar. 4(3):373-84
25. 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.
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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Schwerer B. Antibodies against gangliosides: a link between preceding infection and
immunopathogenesis of Guillain-Barré syndrome. Microbes Infect. 2002 Mar. 4(3):373-84
26. 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.
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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27. 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.
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29. 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.
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Pediatric Guillain-Barré syndrome
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30. 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.
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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31. 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.
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32. Physical Examination (Cont.)
• Occasionally:
o Autonomic instability (26%)
o Ataxia (23%)
o Dysesthesias (20%)
o Cranial nerve findings (35-50%),
predominantly facial palsy (Children>adult)
are noted.
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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Inaloo S, Katibeh P. Guillain-barre syndrome presenting with bilateral
facial nerve palsy. Iran J Child Neurol. 2014 Winter. 8(1):70-2
33. Physical Examination (Cont.)
• 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.
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Pediatric Guillain-Barré syndrome
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34. Physical Examination (Cont.)
• 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.
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Pediatric Guillain-Barré syndrome
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35. Physical Examination (Cont.)
• The autonomic neuropathy involves both
the sympathetic and parasympathetic
systems; manifestations include:
Orthostatic hypotension
Hypertension
Pupillary dysfunction
Sweating abnormalities
Sinus tachycardia
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Pediatric Guillain-Barré syndrome
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37. Diagnosis
The diagnosis of GBS is typically based on
the presence of :
o Progressive ascending weakness
o Areflexia
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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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.
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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39. 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.
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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40. 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),
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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41. 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.
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Pediatric Guillain-Barré syndrome
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42. 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.
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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43. 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
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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44. 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
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Pediatric Guillain-Barré syndrome
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45. 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.
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47. 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
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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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
48. 19/08/2018
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Spinal cord lesions may be considered in the differential
diagnosis:
• Transverse myelitis • Vascular malformations
• Epidural abscess • Cord infarctions
• Tumors • Cord compression
• Enteroviral infections of
the anterior horn cells
• Lumbosacral disk
syndromes
• Poliomyelitis • Trauma
• Hopkins syndrome
49. 19/08/2018
Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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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
50. Treatment
• In pediatrics, the most effective form of
therapy is generally considered to
be intravenous immunoglobulin (IVIG)
• Plasmapheresis may also be used
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51. Prognosis
• In general, the outcome of GBS is
more favourable in children than in
adults
• the recovery period is long, often
weeks to months
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52. 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
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53. 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
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54. Complications of GBS
• The most common serious complications
are:
1. Weakness of the respiratory muscles
2. Autonomic instability
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Ilyas M, Tolaymat A. Minimal change nephrotic syndrome with
Guillain-Barré syndrome. Pediatr Nephrol. 2004 Jan. 19(1):105-6
55. Complications of GBS (Cont.)
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Pediatric Guillain-Barré syndrome
Prof. Dr .Saad S Al Ani
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Ilyas M, Tolaymat A. Minimal change nephrotic syndrome with
Guillain-Barré syndrome. Pediatr Nephrol. 2004 Jan. 19(1):105-6
Other important potential complications
include:
• Pneumonia • Ileus
• Adult respiratory distress
syndrome
• constipation
• Septicemia • gastritis
• Pressure sores • dysesthesias
• Pulmonary embolus • Nephropathy
56. 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
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Pediatric Guillain-Barré syndrome
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57. 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.
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58. 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.
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59. 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
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