gbs journal
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Guillain Barre Syndrome
Journal Reading
Fathia Rachmatina
030.08.099
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Definisi
Guillain Barre syndrome ( GBS ) adalah
suatu kelainan sistem kekebalan tubuh
manusia yang menyerang bagian darisusunan saraf tepi dirinya sendiri dengan
karekterisasi berupa kelemahan atau
arefleksia dari saraf motorik yang sifatnya
progresif. Kelainan ini kadang kadang
juga menyerang saraf sensoris, otonom,
maupun susunan saraf pusat.
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Epidemiologi
The reported incidence of GBS in Western
countries ranges from 0.89 to 1.89 cases
per 100.000 person-years
The incidence increases gradually with
age, but the disease may occur at any
age. Men and women are affected equally
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Etiologi
Infeksi
GBS sering sekali berhubungan dengan infeksi akut non
spesifik. Insidensi kasus GBS yang berkaitan dengan
infeksi ini sekitar antara 56% - 80%, yaitu 1 sampai 4
minggu sebelum gejala neurologi timbul seperti infeksi
saluran pernafasan atas atau infeksi gastrointestinal.
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Patogen yang tersering ditemukan
adalah Campylobacter jejuni, cytomegalo
virus(CMV),Mycoplasmapneumonia,
Epstein-Barr virus, dan virus influenza.
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Gejala Klinis
GBS ditandai dengan timbulnya suatu
kelumpuhan akut yang disertai hilangnya
refleks-refleks tendon dan didahului
parestesi dua atau tiga minggu setelahmengalami demam disertai disosiasi
sitoalbumin pada likuor dan gangguan
sensorik dan motorik perifer.
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Variants
Classical GBS
Recurrent GBS
Miller-Fisher syndrome is characterized bygait ataxia, areflexia, andophthalmoparesis.
Acute sensory polyneuritis
Acute panautonomic neuropathy Acute axonal variant of GBS
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Miller Fisher Syndrome
The Miller Fisher syndrome appears to be
more common among patients with the
GuillainBarr syndrome who live in
eastern Asia than among those who live inother parts of the world
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Most patients with the Miller Fisher
syndrome have evidence of infection 1 to
3 weeks before the development of
ophthalmoplegia or ataxia; in one study,20% of patients had C. jejuni infection and
8% had Haemophilus
influenzae infection.The presence of distalparesthesia is associated with the Miller
Fisher syndrome.
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Diagnosis:
Albuminocytologic dissociation: elevated
CSF protein w/ normal WBC (80-90% pts)
Electromyography (EMG) helps confirm
diagnosis = prolonged or absent F waves
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Patofisiologi
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Terapi
Monitoring cardiac and pulmonary
dysfunction
Prevention of pulmonary embolism
Immunotherapy
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Immunotherapy
Plasma exchange was the first treatment
that was found to be effective in hastening
recovery in patients with the Guillain
Barr syndrome, and it appeared to bemost effective when it was started within
the first 2 weeks after disease onset in
patients who were unable to walk.
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An electrophysiological examination is not
always required for the initiation of
immunotherapy. Plasma exchange
nonspecifically removes antibodies andcomplement and appears to be associated
with reduced nerve damage and faster
clinical improvement, as compared withsupportive therapy alone
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Prognosis
Pada umumnya penderita mempunyai prognosa yang
baik tetapi pada sebagian kecil penderita dapat
meninggal atau mempunyai gejala sisa.
95% terjadi penyembuhan tanpa gejala sisa dalam
waktu 3 bulan bila dengan keadaan antara lain:
pada pemeriksaan NCV-EMG relatif normal
mendapat terapi plasmaparesis dalam 4 minggu mulai
saat onset
progresifitas penyakit lambat dan pendek
pada penderita berusia 30-60 tahun
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