Guillain-Barre Syndrome

Pembimbing :
dr. Kiki Muhammad Iqbal, Sp.S(K)

Penyaji:
1.Jery (190131081)
2.Ulfatul Ulya (190131179)
3.Nur Aisyah Binti Izzudin (190131128)
4.Yolanda Rebecca Tambunan(190131189)
5.Yan Josua Sinaga (190131207)
Outline
• Definisi
• Etiologi
• Epidemiologi
• Klasifikasi
• Diagnosis
• Diagnosis Banding
• Tatalaksana
• Komplikasi
• Prognosis
Definition
• Guillain-Barré syndrome (GBS) is an
acute inflammatory
polyradiculoneuropathy caused by an
autoimmune reaction to the peripheral
nerve.
• The classic presentation is characterized
by an acute monophasic, non-febrile,
post-infectious illness manifesting as
ascending weakness and areflexia.
• With the eradication of poliomyelitis,
GBS is the most common cause of acute
non-trauma-related motor paralysis in
the world. Hauser, S. L., & Josephson, S. A. 2010, Harrison’s Neurology in Clinical Medicine, The
McGraw-Hill Companies, United States.
Epidemiology
• The annual incidence of GBS as reported in one 10-year study was
0.42 cases per 100,000 persons.
• Higher incidence of GBS has been reported in younger adults and the
elderly aged 50 years and older.
• Almost 75% of GBS patients are males, and higher rates of GBS are
reported during the winter and early summer seasons.

Asiri, S., Altwaijri, W., Ba-Armah, D., Al Rumayyan, A., Alrifai, M., Salam, M., & Almutairi, A. (2019),
Prevalence and outcomes of Guillain-Barré syndrome among pediatrics in Saudi Arabia: a 10-year retrospective
study, Neuropsychiatric Disease and Treatment, Volume 15, 627–635. doi:10.2147/ndt.s187994 
Etiology

Asiri, S., Altwaijri, W., Ba-Armah, D., Al Rumayyan, A., Alrifai, M., Salam, M., & Almutairi, A. (2019),
Prevalence and outcomes of Guillain-Barré syndrome among pediatrics in Saudi Arabia: a 10-year retrospective
study, Neuropsychiatric Disease and Treatment, Volume 15, 627–635. doi:10.2147/ndt.s187994 
Etiology
• Evidence from animal models suggests a
key role of molecular mimicry.
• In Campylobacter jejuni gastrointestinal
infections, a lipooligosaccharide present in
the outer membrane of the bacteria is
similar to gangliosides that are components
of the peripheral nerves. 
• Therefore, an immune response triggered to
fight infection can lead to a cross-reaction
on host nerves.

Hauser, S. L., & Josephson, S. A. 2010, Harrison’s Neurology in

Clinical Medicine, The McGraw-Hill Companies, United States.
Postulated immunopathogenesis of GBS associated with C. jejuni infection. Nguyen, T. P., & Taylor, R. S., 2010, ‘Guillain Barre Syndrome’
[Internet] accessed 22 November 2020, Available at:
https://www.ncbi.nlm.nih.gov/books/NBK532254/
 Klasifikasi GBS
JENIS GBS

• Acute Inflammatory Demyelinating Polyneuropathy (AIDP)

 Kerusakan primer pada selubung myelin
 Menyebabkan kelemahan ekstremitas akut dan otot-otot pernapasan
 Menyebabkan gangguan sensorik (kebas dan kesemutan)

• Acute Motor Axonal Neuropathy (AMAN)

 Kerusakan primer pada axon saraf motorik dan sensorik
 Menyebabkan kelemahan akut pada ekstremitas dan otot-otot pernapasan
 Tidak ada gangguan sensorik

• Acute Motor and Sensory Axonal Neuropathy (AMSAN)

 Kerusakan pada axon saraf motorik dan sensorik
 Menyebabkan kelemahan akut pada ekstremitas dan otot-otot pernapasan
 Menyebabkan gangguan sensorik (kebas dan kesemutan)
 Varian
• Miller’s Fisher Syndrome (MFS)
 Terjadi proses dyemielinisasi, dimana antibodi Imunoglobulin G (IgG) merusak gangliosida GQ1b, GD3 dan
GT1a
 Menyebabkan kelemahan akut pada otot-otot mata (ophtalmoplegia)
 Menyebabkan kehilangan keseimbangan dan inkoordinasi (ataksia)
 Menyebabkan hilangnya refleks (arefleksia)
• Ensefalitis batang otak Bickerstaff
 Penurunan kesadaran, ataksia dan oftalmopresis/oftalmoplegia
• Varian Faringeal-servikal-brakial
 Kelemahan pada otot faring, leher atau ekstremitas atas pada awal onset yang kemudian dapat meluas ke
ekstremitas bawah.
 Sering ditemukan Ptosis
• Varian neuropati kranial multipel
 Paresis nervus kranial multipel (nervus II, IV, VI, VII, IX, X, XI)
 Dapat bilateral atau simetris yang kemudian diikuti dengan gejala sensorik dan kelemahan motorik
 Umumnya disertai arefleksia atau hiporefleksia
 Varian
• Diplegia fasial dengan parestesi
 Paresis nervus VII tipe LMN, hiporefleks atau arefleks, tidak ada keterlibatan nervus kranial lainnya

• Paraparesis akut
 Kelemahan terisolasi pada ekstremitas bawah, hiporefleks atau arefleks
• Pandisotonomia akut
 Sangat jarang terjadi
 Gejalanya berupa gejala otonom khususnya pada kardiovaskuler dan visual, kehilangan sensoris
 Diagnosis Guillain–Barré Syndrome
• GBS is considered a clinical diagnosis1
• When to suspect GBS2
– Rapidly progressive bilateral limb weakness and/or sensory deficits
– Hypo/areflexia
– Facial or bulbar palsy
– Ophthalmoplegia and ataxia
– Dysautonomia (Blood pressure or heart rate instability, pupillary dysfunction and bowel or bladder
dysfunction
– Pain (Muscular, radicular, neuropathic)
– In the absence of CNS involvement or other obvious causes
• How to diagnose GBS2
– Check diagnosis criteria
– Exclude other cause
– Consider : 1
Nguyen TP, Taylor RS. Guillain Barre Syndrome. [Updated 2020 Jul
• Routine laboratory tests 10]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls
• CSF examination Publishing; 2020 Jan-
• Electrophysiological studies 2
Leonhard, Sonja E et al. “Diagnosis and management of Guillain-
Barré syndrome in ten steps.” Nature reviews. Neurology vol. 15,11
(2019): 671-683.
Leonhard, Sonja E et al. “Diagnosis and
management of Guillain-Barré syndrome in
ten steps.” Nature reviews. Neurology vol.
15,11 (2019): 671-683.
1. Diagnosis Criteria for GBS

Features required for diagnosis

•Progressive bilateral weakness of arms and legs (initially only one leg may be involved)
•Absent or decreased tendon reflexes in affected limbs (at some points in the clinical course)
Features that strongly support diagnosis
•Progressive phase lasts from days to 4 weeks (usually<2 weeks)
•Relative symmetry of symptoms and signs
•Relatively mild sensory symptoms and signs (absent in pure motor variant)
•Cranial nerve involvement, especially bilateral facial palsy
•Autonomic dysfunction
•Muscular or radicular back or limb pain
•Increased protein level in cerebrospinal fluid (CSF) ; normal protein levels do not rule out the
diagnosis
•Electrodiagnostic features of motor or sensorimotor neuropathy (normal electrophysiology in the
early stages does not rule out the diagnosis)
Leonhard, Sonja E et al. “Diagnosis and management of Guillain-
Barré syndrome in ten steps.” Nature reviews. Neurology vol. 15,11
(2019): 671-683.
Features that cast doubt on diagnosis
•Increased number of mononuclear or polymorphonuclear cells in CSF (>50 x 10 6/L)
•Marked persistent asymmetry weakness
•Bladder or bowel dysfunction at onset or persistent during disease course
•Severe respiratory dysfunction with limited limb weakness at onset
•Sensory signs with limited weakness at onset
•Fever at onset
•Nadir < 24 h
•Sharp sensory level indicating spinal cord injury
•Hyper-reflexia or clonus
•Extensor plantar responses
•Abdominal pain
•Slow progression with limited weakness without respiratory involvement
•Continued progression for >4weeks after start of symptoms
•Alteration of consciousness (except in Bickerstaff brainstem encephalitis)
Leonhard, Sonja E et al. “Diagnosis and management of Guillain-Barré
syndrome in ten steps.” Nature reviews. Neurology vol. 15,11 (2019): 671-683.
2. Laboratory investigations
• Complete blood counts
• Blood glucose
• Electrolytes
• Kidney and liver function
• Testing for preceding infections
• Serum anti-gangliosides antibodies (anti-GM1, anti-GD1A, anti-GT1A and anti-GQ1b)
3. Lumbar puncture to obtain CSF  Mainly to rule out weakness other than GBS
• Classic finding in GBS : Elevated CSF protein level with a normal CSF cell count
(known as albumino-cytological dissociation)
• CSF protein levels could be normal in 30-50% patients in the first week after the
disease onset ; 10-30% in the second week

Leonhard, Sonja E et al. “Diagnosis and management of Guillain-Barré

syndrome in ten steps.” Nature reviews. Neurology vol. 15,11 (2019): 671-683.
4. Electrodiagnostic studies2  in atypical presentation ; also used to
differentiate between AIDP, AMAN and AMSAN
 A sensori-motor polyradiculoneuropathy

5. Imaging (MRI)
• Spine : enhancement of the nerve root  indicating a breakdown of the
blood-nerve barrier due to inflammation in GBS1
• Excluding probability of other causes (Myelitis or intracranial)1,2

1
Nguyen TP, Taylor RS. Guillain Barre Syndrome. [Updated 2020 Jul 10]. In: StatPearls [Internet]. Treasure Island (FL):
StatPearls Publishing; 2020 Jan-
2Leonhard, Sonja E et al. “Diagnosis and management of Guillain-Barré syndrome in ten steps.” Nature reviews.

Neurology vol. 15,11 (2019): 671-683.

Sudulagunta, Sreenivasa Rao et al. “Guillain-
Barré syndrome: clinical profile and
management.” German medical science : GMS e-
journal vol. 13 Doc16. 21 Sep. 2015
Differential Diagnosis of GBS

Jasti AK et al. Guillain-Barré syndrome: causes, immunopathogenic

mechanisms and treatment. Expert Rev Clin Immunol. 2016
Nov;12(11):1175-1189.
mEGOS score in predicting the ability
to walk independently at 6 months

Leonhard, Sonja E et al. “Diagnosis and management of

Guillain-Barré syndrome in ten steps.” Nature reviews.
Neurology vol. 15,11 (2019): 671-683.
Erasmus GBS Respiratory Insufficient Score (EGRIS)

0-2 : low risk of mechanical

intervention (4%)
3-4 : intermediate risk of
mechanical intervention (24%)
≥5 : high risk of mechanical
intervention (65%)
*This model is based on Dutch
population of patients with GBS
(age>6yo) ; has not been validated
internationally

Leonhard, Sonja E et al. “Diagnosis and management of Guillain-Barré syndrome in ten

steps.” Nature reviews. Neurology vol. 15,11 (2019): 671-683.
 Penatalaksanaan GBS
• Sindrom Guillain-Barré berpotensi mengancam jiwa. Penderita GBS harus dirawat di rumah sakit
agar dapat dimonitor secara ketat.
• Perawatan suportif termasuk pemantauan pernapasan, detak jantung dan tekanan darah. Dalam
kasus di mana kemampuan bernapas pasien terganggu, ia biasanya memakai ventilator. Semua
pasien GBS harus dipantau untuk mengetahui komplikasi, yang dapat mencakup detak jantung
yang tidak normal, infeksi, pembekuan darah, dan tekanan darah tinggi atau rendah.
• Tidak ada obat yang spesifik untuk GBS. Tetapi perawatan dapat membantu memperbaiki gejala
GBS dan mempersingkat durasinya penyakitnya.
• Oleh karena GBS merupakan penyakit autoimun, maka fase akut diobati dengan imunoterapi,
seperti pertukaran plasma untuk menghilangkan antibodi dari darah atau imunoglobulin
intravena. Pengobatan lebih efektif bila dimulai 7 hingga 14 hari setelah gejala muncul.
• Dalam kasus di mana kelemahan otot berlanjut setelah fase akut penyakit, pasien memerlukan
layanan rehabilitasi untuk memperkuat otot dan memulihkan gerakan.
Algoritma Terapi
• Treatment Related
Fluctuation : Perbaikan
skala disabilitas GBS
sedikitnya 1 poin setelah
pemberian imunoterapi
diikuti oleh perburukan
skala disabilitas GBS
sedikitnya 1 poin dalam 2
bulan pertama setelah
imunoterapi.
 Komplikasi

Leonhard, Sonja E et al. “Diagnosis and

management of Guillain-Barré syndrome in
ten steps.” Nature reviews. Neurology vol.
15,11 (2019): 671-683.
 Prognosis
• Kebanyakan fungsi pada pasien GBS kembali normal
• Setelah perkembangan penyakit berhenti, gejala biasanya terjadi
selama 2-4 miggu, diikuti dengan pemulihan bertahap.
• Sekitar 20-25% dari pasien memerlukan ventilasi mekanis, dan 5%
meninggal, biasanya dari komplikasi dapat muncul kegagalan
pernapasan atau disfungsi otonom.
• Residual keluhan motorik hadir dalam 25% dari pasien setelah 1
tahun.
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Guillain-Barre Syndrome
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