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kuliah guillain barre syndrome
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Guillain-Barré Syndrome (GBS) and Antecedent Campylobacter
Infection
Muhammad luthfi
Bagian Ilmu Penyakit Saraf Fakultas Kedokteran Universitas Mulawarman
Tahun 2013
Background of GBS
• Definition of GBS
– acute demyelinating disorder, primarily involving the peripheral nervous system (PNS)
– degree and location of disability depends on the myelinated nerves involved: MOTOR
Background of GBS• History of GBS
– 1830s - 1st clinical descriptions
– 1859 - Landry -- detailed description (Landry’s paralysis)
– 1916 - Guillain, Barré and Strohl - disease accompanied by “hyperalbuminosis”
in CSF
– 1936 - Guillain - cardinal clinical features
– 1950s - Electromyography
– 1969 - Asbury - inflammation is quintessential feature of GBS
Background of GBS
• Epidemiology of GBS
– annual incidence is fairly uniform through the world.
– 1-4 cases/100,000
– more common in males
– increases with age
– seasonal variation in incidence
Background of GBS• Clinical Features of GBS
– Motor Involvement*• “ascending paralysis”
• distal (arms/legs) to proximal
– Sensory Involvement
– Autonomic Involvement• principal cause of mortality
Background of GBS• Clinical Features of GBS
– signs and symptoms depend on the part of the nervous system involved
– usually progressive and peaks in 1-3 weeks
– most regain lost function, but time for recovery variable and depends on severity (6 weeks- 2 years)
Background of GBS
• Clinical Feature of GBS
– Atypical presentations
• Miller-Fisher Syndrome– Areflexia
– Ophthalmoplegia
– Ataxia
Background of GBS
• Diagnosis of GBS
– Patient history-- previous illness or vaccination
– Clinical presentation (Physical Exam)
– Laboratory Evaluation• Increase in protein in CSF
• Electromyography -- nerve conduction block
Background of GBS
• Treatment of GBS
– supportive care
– plasmapheresis
– corticosteroids and immunosuppressants
– artificial ventilation (if necessary)
Background of Campylobacter
• Leading cause of bacterial diarrhea in USA
• Campylobacter jejuni - principal human pathogen
• Microbiology– Gram negative, S-shaped rod
– monotrichous flagella
– many serotypes
Background of Campylobacter• Scanning electron microscope image of
Campylobacter jejuni
• Image taken from Altekruse et al. Campylobacter jejuni - an emerging foodborne pathogen. EID. 5(1):1999
Background of Campylobacter• Epidemiology of Campylobacter
– estimated 200,000-400,000 cases/yr???
• Image taken from Altekruse et al. Campylobacter jejuni - an emerging foodborne pathogen. EID. 5(1):1999
Background of Campylobacter
• Epidemiology
– mode of transmission: fecal-oral• vehicles - food, water, milk
• direct contact
– reservoirs: • poultry and cattle
• domestic pets, swine and humans
Background of Campylobacter
• Clinical features
– asymptomatic
– symptomatic • acute watery diarrhea
• usually self-limited
Pathogenesis of GBS
• Believed to have autoimmune etiology
Presence of anti-ganglioside IgG in blood
?
Loss of myelin surrounding nerves
Antecedent Events of GBS• Evidence from case control studies
suggest association with antecedent events:
– Campylobacter jejuni
– Cytomegalovirus
– Epstein-Barr virus
– Mycoplasma pneumoniae
– Rabies vaccine
– “Swine flu” vaccine
Campylobacter and GBS• Prevalence of prior C.jejuni infection varies
geographically:– 66% in China --4% in USA
• Increased incidence in GBS in China parallels seasonal increase in Campylobacter infections
• Exceptional report of 2 sisters who developed GBS at the same time following C. jejuni infection of the Penner serotype 0:19
Campylobacter and GBS
• Only 1 in a 1000 cases of symptomatic
C. jejuni enteritis is followed by GBS
• Uncommon strains of C. jejuni are often isolated from GBS patients– Penner serotype 0:19 (China, Japan)– Penner Serotype 0:41 (South Africa)
Campylobacter and GBS• Molecular mimicry hypothesis
– Host immune response to foreign antigen cross-reacts with host tissue resulting in the immune attack of nerve myelin
– Immunoglobulins, usually IgG, are elevated in GBS
– Host target antigen is unknown
(? gangliosides)
Campylobacter and GBS• Evidence for antibodies directed toward
host gangliosides in GBS patients
– gangliosides
• are complex glycospingolipids with sialic acid residues
• distinguished by # and position of sialic acid residues
• concentrated in neuronal membranes and other cells, BUT are in low concentrations in myelin
Campylobacter and GBS
• C. jejuni has GM1-like epitopes on its membranes
• Anti-ganglioside GM1 antibodies are present in 14-50% of GBS patients
• Only 50% of patients with GBS and anti-ganglioside antibodies have evidence of preceding C. jejuni infection
Campylobacter and GBS
• Fisher syndrome
– IgG1 antibodies to ganglioside GQ1b present– Highly specific for disease– Strongest evidence for autoimmune etiology,
but is not commonly preceded by C. jejuni infection