30/05/2012

GUILLAIN-BARRE SYNDROME

Tun Paksi Sareharto

History
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Waldrop 1834 Brussel’s Conf. 1937

Olliver 1837 Haymaker & Kernohan
1949
Landry 1859
Waksman & Adams
Graves 1884 1955
Ross & Bury 1893 Miller Fisher 1956
Guillain, Barre Asbury, Aranson &
& Strohl Adams 1969
1916-1920
Georges Guillain

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Revue Neurologique 1916

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What is the GBS?

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Thomas Munsat 1965: “…The GBS is easy to diagnose but

difficult to define

Definisi:

Proses inflamasi non infeksi dengan ditandai infiltrasi limfosit
dan makrofag ke makrofag ke radiks serabut syaraf tepi,
disertai destruksi selubung mielin secara segmental oleh
karena proses autoimun.

Epidemiologi
Insidensi 0,25 – 1,5 per 100.000 anak usia < 16 tahun.
Laki-laki : perempuan :1,5 : 1.

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Patogenesis
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Infeksi virus menyebabkan penurunan kadar supresor

sel T sehingga terjadi peningkatan sel T dan sel B
serta limfosit

Kontak antara sensitive lymphocyte dengan serabut
saraf
kerusakan myelin

Cellular & Humoral Immune

6 Mechanisms

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Antecedent Events: Infectious

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Viral: Influenza, Coxsackie, EBV, Herpes,

HIV, Hepatitis, CMV, WNV
Bacterial: Campylobacter jejuni,
Mycoplasma, E. coli
Parasitic: Malaria, Toxoplasmosis

Antecedent Events: Systemic disease

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Hodgkins

CLL

Hyperthyroidism

Sarcoidosis

Collagen Vascular d.

Renal d.

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Other antecedent events

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Surgery

Immunization

Pregnancy

Envenomization

Bone marrow transplantation

Drug ingestion

Manifestasi Klinis
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50-60% di dahului infeksi sal nafas atau sal cerna 4

minggu sebelumnya.

Kelumpuhan motorik yang progresif bersifat
asenderen dan simetris.

Awalnya mengenai kedua tungkai bawah dalam
waktu 2-4 minggu menyebar keatas menimbulkan
kelemahan otot yang lengkap

Kelumpuhan flaksid, reflek fisiologis menurun, refleks
patologis negatip

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Manifestasi Klinis
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Proses kelumpuhan mencapai puncak dlm 4 minggu,

menetap selama 2 mgg kemudian terjadi
penyembuhan

Gejala klinisnya meliputi

1. fase progresif selama 10 -12 hari

2. fase plateau 10 -12 hari

3. fase pemulihan selama beberapa minggu atau
beberapa bulan.

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Manifestasi Klinis
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Kelumpuhan otot muka, asimetri ( 20 -50%)

Gangguan bulbar (25%), kelumpuhan syaraf
okulomotor ( 8 -10%).

Gangguan vasomotor : hipertensi, hipotensi.

Gangguan sensoris pada 2/3 kasus ( parestesi,
hipestesi)

Autonomic dysfunction.

The typical illness evolves over weeks usually

following an infectious disease and involves:
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1. Paresthesiaes
3. Diminution and

usually hearld the loss of the DTRs
disease

4. Albuminocytologic

2. Fairly symmetric dissociation
weakness in the legs,
later the arms and,
5. Recovery over
often, respiratory and weeks to months
facial muscles

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Diagnosis
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Kelumpuhan motorik progresif mengenai lebih dari 1

ekstremitas.
Penurunan refleks tendo dengan berbagai derajat.

Kelumpuhan progresif berhenti, puncaknya sampai 4 mgg
simetris disertai gangguan sensoris, syaraf kranial, disfungsi
otonom.

Tidak ditemukan demam.

CSS : dissosiasi sito albumin (peningkatan kadar protein
(100-1000 mg/ml) tanpa pleositosis (peningkatan jumlah sel)
setelah 1 minggu pertama sampai 3 minggu timbulnya gejala
penyakit

Adanya abnormalitas konduksi syaraf (80%) pada pem EMG.

Tidak adanya bukti bahwa kelumpuhan otot karena keracunan
hexacarbon porphyria/ peny diphteria.

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Differential Diagnosis
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Consider the possibility of an upper motor neuron

lesion

Other considerations are rare. Diphtheritic neuritis &
poliomyelitis belong more to the history section of
this presentation. A new possibility is West Nile
Virus.

Poliomyelitis

Miositis akut

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Differential
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N-M: MG, LES, Antibiotics

Toxic: Cigutera (ciguatoxin), Pufferfish
(tetrodotoxin), Shellfish (saxitioxin), Botulism, Tick
paralysis (Lone Star tick, Gulf Coast tick), Glue
sniffing, Buckthorn

Mononeuritis multiplex assoc. c Wegner’s. PAN, SLE,
RA, Sjogren’s, Cryoglobulinemia etc.

Differential
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Metabolic: Periodic paralyses, Hypokalemia,

Hypermagnesemia, Hypophoshatemia c parenteral
hyperailimentation, Thyrotoxicosis, ICU
myoneuropathy (CIP)

Heavy metal: Lead, Arsenic, Thallium, Barium c
hypokalemia

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Penatalaksanaan
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Pemantauan ketat
gangguan pernafasan

Plasmapheresis pada anak dengan berat badan diatas 15
kg

Gamaglobulin (iv) 0,4 gr/kgBB/hr selama 5 hari

Steroid tidak dianjurkan.

Treatment
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Respiratory failure
Autonomic dysfunction
DVT & PE
Pain
Positioning & Skin care
Physical therapy
Nutrition

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Respiratory Failure
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Oropharyngeal weakness in ~25% with impaired

swallowing of secretions & aspiration

Mechanical respiratory failure- mainly due to
diaphragmatic weakness (Phrenic nerves.)
Inspiratory c MIF (Max. Inspir. Force) a good
supplement measure to FVC

Respiratory Failure
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~33% require intubation

Avg. time to intubation is 1 week & these pts. have
substantially longer recovery time

Need is unlikely if patient does well for 2 wks. post
onset of paresthesiaes

Guidelines: FVC <15 mL/kgm
MIF < 25 cm water

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Psychological
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Fear

Helplessness

Communication

Pain

Sleep deprivation & hallucinosis

Depression

Visits from other GBS patients

Corticosteroids
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Lancet 1993 242 pts.

IV Methylprednisilone 500 mgm/day x 5.

Ineffective
May cause relapse

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Plasma Exchange
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Removal of the blood’s liquid soluble components

including complement, immunoglobulin, immune
complexes, cytokines and interleukins

A typical session removes about 60% of the body
mass of plasma proteins which is replaced c saline,
albumin & FFP

Done qod for 3-5 sessions

Plasma Exchange
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Various studies since 1985

Time on ventilator reduced by ½

Full strength regained at 1 year: Exchange 71%,
Untreated 52%

Limitations: Limited availability
Avoid with autonomic instability

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IVIG
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Mechanism of action- unknown

? Antiidiotypic antibody action
? Inhibition of cytokines
? “Sponging” of complement
? Binding to Fc receptors so macrophages
can’t bind

IVIG
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Dosage: 0.4 gms/kgm/day x 5 c each dose given

over 3-4 hours preceded by IV diphenhydramine
&/or po ibuprofen

Caution c renal insufficiency or IgA deficiency

38 Center trial in 1997

Equal to plasma exchange

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Prognosis
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2-12% die of complications

75-85% have good recovery

15-20% have moderate residual deficits

1-10% left with severe disability

Worse outcomes if older age, poor UE strength, need for
ventilator support, rapidly progressing weakness, CSF levels of
high molecular weight monofilament protein

GBS is a life changing event with potential long lasting
influence

Prognosis in Kids
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Childhood mortality rate < 5%

Death usually from respiratory failure

Most common serious complication is weakness of respiratory
muscles and autonomic instability

Most (90-95%) kids recover in 3-12 months

5-10% have significant permanent disability

Outcome usually better than for adults

Recovery periods lasts longer than acute illness

5% recurrence rate

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