2. OBJECTIVES:
• Introduction
• Epidemiology
• Etiology
• Pathogenesis
• Clinical features
• Variants of GBS
• Investigation and diagnosis
• Treatment
• Prognosis
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3. INTRODUCTION:
• Guillain-Barré syndrome is a rapidly
progressive, autoimmune,
polyradiculoneuropathy, predominantly motor,
leading to ascending symmetric paralysis,
respiratory compromise and autonomic
dysfunction, triggered by preceding infection.
• It is a common cause of acute , acquired
weakness in children.
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4. EPIDEMIOLOGY
• In the post-polio era, GBS is the most common
cause of acute flaccid paralysis in children.
• Occurs worldwide with an incidence of 0.34 to
1.34 cases per 100,000 persons aged ≤ 18 years.
• Incidence is lower in children than adults.
• Incidence increases by approx. 20% with every 10
year increase in age.
• Males > females by 1.5 times in all age groups.
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5. Etiology
• Weakness of limbs usually follow a non-specific
viral infection by about 10-15 days.
• The infection might have caused by :
– GI tract: Campylobacter jejuni, Helicobacter
pylori
– Respiratory tract: Mycoplasma pneumonia
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7. PATHOGENESIS
• Myelin sheath insulate the nerve
and allows electrical signal to travel
along it.
• Syndrome often begins from
days/weeks after flu like illness or with gastro
intestinal symptoms.
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8. 8
Entry of Infective agents (C. jejuni )
Via MHC II APC presents Antigen to CD4 cells and B cells
Production of cytokines and interleukins occurs Activation of
macrophages
More cytokines and interleukins recruits more macrophages.
B cells produces antibodies
Macrophages uses those antibodies to neutralize the
offending agents.
Accidental binding to epitopes present on surface of myelin
sheath due to sharing of similar molecular lipids that antigens
have. (MOLECULAR MIMICRY)
9. • Though everyone getting infected with Campylobacter
Jejuni doesn’t develop GBS hence some other
pathophysiology is yet to be discovered.
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That leads inflammatory changes causing deterioration of
myelin sheath due to production of Ganglioside antibodies.
Demyelination happens in the patches along the length of
axon
In early state of GBS remyelination occurs but over the time
schwann gets irreversible changes.
Disruption of electrical impulse transmission through axon.
End up getting muscle weakness, alter sensation and
ultimately paralysis.
11. CLINICAL FEATURES
• Paresthesia :
– altered/loss of Senstaion (gloves and stocking )
– Mostly affects nerves that convey vibration and touch
sensation
• When motor nerves supplying muscles are
affected:
– Muscle weakness and absence of reflexes occurs
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12. – Lower extremity symmetric or modestly asymmetric
weakness that may ascend over hours to days to involve
trunk and arms.
• Cranial nerves involved (bulbar palsy):
– Double vision , facial weakness, dysphagia with pooling of
secretions.
• Nerves supplying respiratory muscles like the
diaphragm:
– May need mechanical ventilation , or leads to death.
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13. • Affected children are irritable, there is refusal to walk
and gait abnormalities.
• Autonomic dysfunction :
• Cardiac dysrhythmias (bradycardia, persistent sinus
tachycardia, atrial and ventricular tachyarrhythmia)
• Orthostatic hypotension
• Abnormal sweating
• Constipation
• Urinary incontinence
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14. VARIANT OF GUILLAIN-BARRÉ SYNDROME
1. Acute inflammatory demyelinating
polyneuropathy (AIPD):
– Most common form of GBS
– It accounts for about 85-90% of cases.
2. Acute motor axonal neuropathy (AMAN):
– It is more common in developing nations
– Has a seasonal incidence and is associated with a
preceding Campylobacter jejuni infection.
– Clinical features and recovery are similar to AIPD
however it is more severe form.
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15. 3. Acute motor-sensory axonal neuropathy
(AMSAN):
– It resembles the motor axonal variant but has more
sensory symptoms
– Pathology is predominantly axonal lesions of both
sensory and motor nerve fiber.
4. GQ1b syndrome:
– Some forms has features of eye movement abnormalities
and ataxia rather than limb weakness.
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16. – These forms are associated with frequent
seropositivity to antibodies against the GQ1b
ganglioside and includes MFS, Bickerstaff encephalitis
and pharyngeal-cervical-branchial weakness
5. Miller Fisher syndrome (MFS):
– Characterised by external opthalmoplegia, ataxia,
muscles weakness and areflexia.
– CSF analysis features are similar to that of (AIDP).
GQ1b antibodies are frequently found in patients with
MFS
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17. INVESTIGATIONS AND DIAGNOSIS
Initial diagnosis is based upon clinical presentation
and typical features.
1. CSF analysis : elevated CSF protein (> 45 mg/dl)
with normal CSF WBC count (Albuminocytologic
dissociation)
2. Electrodiagnostic studies :
a. Motor conduction velocities are greatly
reduced and slowing of sensory conduction .
b. Electromyogram shows evidences of acute
denervation of muscles
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18. 3. Magnetic resonance imaging: Spinal MRI
with contrast shows enhancement of the
ventral nerve roots (often) and dorsal nerve
roots (less often).
4. Serological testing for campylobacter jejuni,
Mycoplasma infection is also helpful.
5. Serum creatine kinase (CK) level may be
mildly elevated .
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19. 6. Histopathology :
• The dominant histopathologic finding is
inflammation of peripheral nerves
• manifested as perivenular and endoneurial
infiltration of lymphocytes, macrophages and
a few plasma cells.
• Segental demyelination is seen.
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20. 7. Antibodies :
• Acute demyelinating form of GBS is caused by
immune reaction directed against epitopes in
cell surface of Schwann cell or myelin causes
axonal forms of GBS.
• In clinical practice, commercially available
testing for serum IgG antibodies to GQ1b is
useful for the diagnosis of Miller Fisher
syndrome.
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21. TREATMENT
1. Immunotherapies :
• Main modalities of therapy for GBS are
intravenous immune globulin (IVIG) and
plasma exchange (Plasmapheresis).
• Indications for immunotherapy:
– Progressing weakness
– Worsening respiratory status or need for
mechanical ventilation
– Significant bulbar weakness
– Inability to walk unaided.
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22. • IVIG:
– total dose of IVIG is 2g/kg
– given as 1g/kg for two days or 400 mg/kg for 5
days who are unable to walk unaided if presented
with in 2-4 weeks of onset of symptoms.
• Plasma exchange is for those who are unable
to walk unaided if treatment is started or
presented with in 4 weeks of symptoms onset.
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23. 2. Autonomic status:
• Close monitoring of blood pressure, fluid
status, cardiac rhythm and sphincter status.
3. Supportive measures:
• Mechanical ventilation if respiratory failure
• Orogastric/nasogastric tube feeding,
gastrostomy or parenteral nutrition is often
necessary.
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24. • Prevention of bed sore , frequent changing
positions and proper nursing care.
• Prevention of DVT
• Bowel and Bladder care
• Physiotherapy
• Glucocorticoids are not beneficial for GBS and
have no role in its treatment.
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25. Prognosis
• In general prognosis in children is better than in
adults.
• Mortality is generally < 2 to 3 % in most pediatric
GBS.
• Approx. 90% of children were ambulatory within
in six months and nearly all (96%)walks within 1
year.
• Recurrence of GBS was reported in 2 to 5 % which
can occurs months to years after the initial episode.
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26. • Although most children experience excellent
recovery but outcome may be less favorable in
the following groups :
– Very young age (< 2 years)
– Severe weakness at presentation
– Rapid progression of weakness
– Cranial nerve involvement
– Required ventilator support
– Have inexcitable motor nerves
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