2. •Guillain-Barré syndrome is a postinfectious
polyneuropathy involving mainly motor but
sometimes also sensory and autonomic nerves.
•Body's immune system begins to attack the body
itself, causing autoimmune disease. In this
syndrome, the immune system starts to destroy
the myelin sheath that surrounds the axons
nerves.
3.
4. Epidemiology
• The incidence <18 years is 0.34 to 1.34 cases per
100,000/year
• It can occur at any age, The average age is 4 to 8 years
and is rare below the age of 2 years
• Males more susceptible
• More common in devloping countries.
• Germany 7.9% ,Mexico (44%) ,India (28%).
• Emerged as the major cause of AFP.
• Immunization with the H1N1 vaccine in 2009 resulted in an
increased incidence of 0.8 cases per million doses
5. Etiology
• Campylobacter jejuni, Helicobacter pylori, Mycoplasma pneumoniae
• West Nile virus,Epstein-Barr Virus,Echo Virus,Coxsakie Virus,Influenza
Virus
• Mumps,Measles
• Vaccines against rabies,influenza, poliomyelitis (oral), conjugated
meningococcal vaccine particularly serogroup C and recently with
H1N1.
• Some Surgery
6. Mechanism of Development of GBS
•Exogenous triggers are believed to activate T
cells, which act against a variety of specific
endogenous antigens(myelin),Resemblance of
the triggering pathogens to antigens on
peripheral nerves leads to activation of
autoimmune response mounted by T
lymphocytes in cooperation with B lymphocyte
7.
8. Clinical Features
• Initially Pain in the muscle
• Weakness of muscle
-The onset is gradual and progresses over days or weeks.
-By the 3rd week 90% of the patient are weak.
-Usually begins in the lower extremities and progressively involves
the trunk, the upper limbs, and finally the bulbar muscles.
-This pattern is known as Landry ascending paralysis.
-Relatively symmetrically,but asymmetry is found in 9% of patients
-Paresthesias occur in some cases.
9.
10. • Respiratory insufficiency can result due to Intercostal
and diaphragmatic muscle paralysis
• Dysphagia and facial weakness are often impending
signs of respiratory failure. They interfere with eating
and increase the risk of aspiration
• Papilledema is found in some cases, oculomotor and
other cranial neuropathies are severe
11. •The autonomic nervous system is also
involvement causing lability of blood pressure
and cardiac rate, postural hypotension, episodes
of profound bradycardia, and occasional
asystole occur.
12. Diagnosis
•History
•Affected children are irritable
•Unable or refuse to walk and later to flaccid
tetraplegia
•In the early course Tendon reflexes are lost but
sometimes preserved until later.Planter reflex is
normal.
13.
14.
15. Diagnosis
1 CSF examination
Albumin-Cytologic Dissociation
The albuminocytologic dissociation in a patient with an acute or subacute
polyneuropathy is diagnostic of Guillain-Barré syndrome.
Usually seen after 48 hours of onset
2 Motor NCVs
• Motor NCVs are greatly reduced, and sensory nerve conduction time is
often slow.
3 MRI of the spinal cord
•Thickening of the cauda equina and intrathecal nerve roots
16.
17. • Antiganglioside antibodies
Antiganglioside antibodies,mainly against GM1 and GD1, are sometimes elevated
• Serum creatine kinase (CK) level may be mildly elevated or
normal.
• Electromyography (EMG) shows evidence of acute
denervation of muscle.
• Serologic testing for Campylobacter and Helicobacter
infections
• Sural nerve biopsy tissue shows segmental demyelination,
focal inflammation and wallerian degeneration
18. Treatment
• There is no known cure for Guillain-Barré syndrome.
• In a patients with slow progression closely monitered to
prevent respiratory failure and respiratory arrest
• Supportive care, such as respiratory support, and treatment
of secondary bacterial infections
• Rapidly progressive ascending paralysis IVG 0.4gm/kg/day for
5 consecutive day
• Plasmapheresis and/or immunosuppressive drugs are
alternatives if IVIG is ineffective.
19. •Combined administration of immunoglobulin
and interferon is effective in some patients.
•Respiratory paralysis may necessitate
tracheostomy and assisted ventilation.
•Patients need tube feeding, care of bowel &
bladder & frequent change of posture to prevent
bedsores
•Physiotherapy is done to prevent handicaps
22. Prognosis
• Usually benign, and spontaneous recovery begins within 2-3
wk.
• Most patients recover within 1-6 months without functional
disability, with 80% of patents having complete recovery
within 12 months.
• The mortality associated with GBS is approximately 3%.
• Mortality is 10-20% from respiratory insufficiency.
• The most common causes for death are autonomic
disturbances.