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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