SYNONYMS : GULLAIN-BARR SYNDROME

1: Idiopathic Polyneuritis
2 : Acute Febrile Polyneuritis
3: Infective Polyneuritis
4: Post Infectious Polyneuritis
5 : Acute Inflammatory Demyelinating
Polyradiculoneuropathy

6 : Gullain-Barr Strohl Syndrome

7 : Landry Ascending Paralysis
8 : Landry Gullain-Barr Syndrome
1

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Dahulu, SGB ini diduga disebabkan oleh infeksi virus.
Akan tetapi akhir-akhir ini terungkap bahwa ternyata virus
bukan sebagai penyebab. Teori yang dianut sekarang
adalah adanya suatu kelainan immunobiologic baik secara
primary immune response maupun immune mediated
process.
Adapun infeksi sebelumnya yang dapat sebagai pencetus
terjadinya SGB adalah: infeksi campylobacter jejuni
( infeksi pada gastrointestinal), immunisasi, kehamilan
ataupun pembedahan.

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Bagaimanakah timbulnya demyelinisasi
segmental?(Neurologi)
Penyakit ini diduga disebabkan adanya
sensitisasi limfosit terhadap antigen
syaraf perifer. Dijumpai demyelinisasi
yang difus, tidak sempurna dan
segmental dari saraf perifer

Myelin Aids Peripheral Nerve

Signal Conduction

In GBS, the most common part

of the nerve initially damaged is the myelin. Myelin acts like the
insulation
on electrical household wires, and assists rapid and accurate signal
conduction, preventing the axon signal from short circuiting or
slowing

Saltatory conduction

Immunobiolog
y of GBS

Doorn 4/27/16
PA,et.al. Lancet Neurolo 2008;7:939-50
.

Cellular & Humoral Immune

Mechanisms
-GBS is believed to result from an autoimmune
response, both humoral and cell mediated
-Its relation to antecedent infections and the
identification of various antiganglioside
antibodies suggest that molecular mimicry may
serve as a possible mechanism for the disease.
The antibodies formed against gangliosidelike
epitopes in the lipopolysaccharide layer of some
infectious agents have been shown in both
necropsy and animal models to cross-react with
the ganglioside surface molecules of peripheral
nerves.
-Symptoms generally coincide pathologically
with various patterns of lymphocytic infiltration
and macrophage-mediated demyelination,
depending on the subtype in question

PATHOGENESIS

-Trauma
-Entrapment
-Tumor

-GBS
-Metabolic
-Nutritional

Dying Back

- Toxin
- Nutritional

PATHOLOGICAL TYPES OF GULLAIN-BARR

SYNDROME
GBS

AIDP
(DEMYELINATI
NG)
AIDP +
Secondary
Degeneration

AXONAL
PATTERN

MILLER
FISHERS
SYNDROME

AMAN
AMSAN
(Acute Motor
Sensory Axonal
Neuropathy)
PIN PERDOSSI,
SOLO, 6-8
NOVEMBER 2014

11

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Bagaimanakah kriteria diagnosis dari SGB?
Kelumpuhan yang flaksid, difus, simetris, sering disertai
kelumpuhan fasialis bilateral
Gejala sensorik kurang menonjol dibandingkan gejala
motorik
Remisi sempurna terjadi dalam 6 bulan, hampir pada
semua kasus
Kenaikan kadar protein CSF (0,55 g/l) dengan jumlah sel
normal atau sedikit meninggi ( terjadi minggu ke 2
paralise)
Tidak ada demam atau hanya sedikit kenaikan suhu badan
sewaktu timbulnya kelumpuhan.
Leukosit normal atau limfosit

CLINICAL SYNDROME of GBS

Acute onset and Progressive course Weakness of LMN type
: - hyporeflexy or areflexy of peripheral cranial / spinal
nerves
Preceded with paresthesia, 2-3 weeks after episode of
fever
CSF Feature : Albuminocytologic Dissociation
(Normal Cell , Albumin content)
Electrophysiology study :
NCV and Distal Latency
_ Reflex / Absent

AMAN :
Pure motor Axonal Subtype
Onset to 1st Exam < (more rapidly) (<AIDP)
Onset to PEAK of clinical syndrome > rapid

13

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Bagaimana prosedur penegakan
diagnosa SGB?
Prosedur diagnostik SGB :
1. Analisis LCS: disosiasi
sitoalbumin
2. EMG

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Bagaimana hasil pemeriksaan cairan
serebrospinalis pada SGB?
Pemeriksaan cairan serebrospinal pada
SGB didapatkan kadar protein yang tinggi,
kadang-kadang dapat sampai 1.000 mg%,
hal ini tidak sesuai dengan jumlah sel
yang ada dalam cairan serebrospinal.
Keadaan ini disebut sebagai disosiasi
sitoalbumin yang mencapai puncaknya
pada minggu ke 4-6

ELECTROPHYSIOLOGY
STUDY
AIDP (DEMYELINATION)

PIN PERDOSSI, SOLO, 6-8

NOVEMBER 2014

16

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Sebutkan yang termasuk diagnosa banding
dari SGB? (Neurologi)
Diagnosa banding SGB :
-Poliomyelitis
-Miastenia Gravis
-Lyme disease
-Periodik paralisis
-Botulismus akut
-Myelitis transversalis Akut

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Bagaimana pengobatan SGB?
Pengobatan SGB :
- Terapi umum : Perawatan untuk mencegah dekubitus, pemasangan kateter urin jika
terjadi retensi urine, fisioterapi, bantuan pernafasan

-Terapi spesifik :
A. Kortikosteroid
-Prednison 15 mg/ hari 4 kali selama 1 minggu,
-Methyl prednisolon 500 mg intravena sehari sekali selama 5 hari
B. Plasmapharesis dosis 200 250 ml/kg BB dalam 4-6 x pemberian.
C.Imunoglobulin intravena : IVIG dosis 0,4 gr/ kgBB/ hari selama 5 hari
(pilihan yang terbaik).

Factors suggesting need for endotracheal intubation in

gbs patients

Forced vital capacity (FVC) < 15

cc/kg
Negative inspiratory force (NIF) < -20
cm H2O
Inability to clear secretions (concern
for aspiration)
Use of accessory muscle for
breathing

First-line Therapies :
Advantages and Disadvantages
Therapy

Advantages

Disadvantages

IGIV

-Early results.
-Class I evidence
supports use

-Expensive, invasive
-Some contraindications, AEs
- Short-lived efficacy.

Corticosteroi
ds
-

-Inexpensive
-Easy to administration

Serious AEs with long-term use

and tolerability issues.
May cause relaps.
Class II evidence only.

-Early results.
-Class I evidence
supports use.

Expensive, invasive, limited

availibility.
Some contraindications, AEs
Short-live efficacy

Methylprednisolo
n 500 mg/day ( 5
days)

Plasma
Exchanges

Comparing PLEX and IvIg

TPE
Early response.
Predictable.
Low risk of renal failure.
Procedure carries no
risk of unknown
infection.
Provides proof of
concept of antibody
mediated disease.
Proven efficacy in CNS
diseases (NMO, acute
demyelination).
Complications largely
preventable.

IvIG
1-2 weeks of delay.
Variable response.
Risk exists in IgA
deficiency.
Not necessarily safe for
new or emerging
infections.
Made of immune
modulation not direct.
CNS efficacy uncertain
(BBB penetration ).
Unpredictable
complication (e.g.
sterile meningitis ).

Hughes RA, Swan AV, van Doorn PA. Intravenous

immunoglobulin for Guillain-Barr syndrome.
Cochrane Database Syst Rev. 2014 Sep

PLASMA EXCHANGE

4/27/16

ourtesy of dr. Nortinah

24

Principle of procedures
(SPECTRA OPTIA)

SEPARATION BY CENTRIFUGATION

Centrifugation
Separation based on
specific gravity
Continuous flow

Process of Therapeutic
Apheresis
Drawn

2
6

Centrifu
ge

Collectio
n

Process
Monitor

Function of Therapy
1.1.Guillain-Barr
syndrome (GBS)
Sickle Cell
Disease
Thrombocytosis:
> 500,000 WBC*or
1.
Cartilage
deffect
Leukocytosis:
> 100,000/L
Neutropenia
2.2.1,000,000/L
Myasthenia
Gravis
Malaria
platelets*
2. Leukemia

Theurapetic Plasma
Exchange

Plasma

Platelet
Depletion
WBC
Depletion

Platelet
White Blood
Cell

RBC

2
7

Limfosit
Monosit
Granulosit

MNC Collection
Granulocyte
Collection

RBC Exchange

Neurologic
Disease

Category

Acute/chronic inflamatory demylinating

polyradiculoneuropath

Lambert-Eaton myasthenia

II

Multiple Sclerosis

III

Myasthenia Gravis

Acute CNS inflammatory demylinating

II

Demylinating polyneuropathy IgG and

IgA

Paraneoplastic neurologic syndrome

III

AABB/ASFA Guidelines
Category I:

Considered primary or standard therapy usually on basis of controlled trials

Category II: Supportive or adjunctive to other therapy

Category III: Insufficient data to determine effectiveness; results of clinical trials may be
conflicting or uncontroled anecdotal reports of efficacy
Category
4/27/16 IV: do not respond to apheresis therapy