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Guillain-Barre Syndrome
Guillain-Barre Syndrome
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Guillain-Barré Syndrome
is an acute inflammatory
demyelinating polyneuropathy
(AIDP), a disorder affecting the
peripheral nervous system. It is
usually triggered by an acute
infectious process.
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Synonim:
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Epidemiology
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Etiology
Autoimmune disease, self limitted
2/3 of patients are preceded by symptoms of upper respiratory tract
infection or gastroenteritis.
Guillain-Barré syndrome has been reported to follow
– vaccinations
– epidural anesthesia
– thrombolytic agents
• It has been associated with some systemic processes, such as
– Hodgkin's disease
– SLE
– Sarcoidosis
– infection with Campylobacter, Lyme disease, EBV, CMV, HSV,
mycoplasma, and recently acquired HIV infection
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Pathogenesis
Peripheral nerve demyelination in Guillain-Barré
syndrome is believed to be immunologically
mediated
Humoral factors and cell-mediated immune
phenomena have been implicated in the damage of
myelin and/or the myelin-producing Schwann cells
Loss of the myelin sheath in Guillain-Barré syndrome
makes nerve impulse transmission is aborted.
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Pathogenesis
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History
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Progresses over no more than 4 weeks
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Clinical Features
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Motor symptoms
Symmetrical limb weakness initially be proximal > distal muscles or
global
Usually the lower extremities before the upper (ascending paralysis)
Trunk, intercostals, neck, bulbar and cranial nerves may be affected
later
Respiratory muscle weakness (25%)
The trunk and cranial nerves may become involved.
If there is cranial nerve involvement, cranial nerve VII, the facial nerve, is most
often affected.
Areflexia
Guillain-Barré syndrome does not affect level of consciousness, pupillary
function, or cerebral function.
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Sensory symptoms
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Autonomic dysfunctions
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Monitoring
Neurological examination
Complaints of dyspnea
Cardiac monitor
Blood pressure
Vital capacity
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DIAGNOSTIC STUDIES
Electrophysiologic studies(EMG/ENMG) are the most
specific and sensitive tests for diagnosis of the disease
They demonstrate a variety of abnormalities indicating
evolving multifocal demyelination
– Slowed nerve conduction velocities
– Partial motor conduction block
– Abnormal temporal dispersion
– Prolonged distal latencies
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DIAGNOSTIC STUDIES
After the first week of symptoms, analysis
of the cerebrospinal fluid (CSF) typically
reveals High protein with normal cell count (cito-albuminic dissociation )
– normal pressures
– few cells (typically mononuclear)
– an elevated protein conc. (greater than 50 mg/dL)
• Early in the course (less than one week), protein levels may not yet be
elevated, but only rarely do they remain persistently normal
• If CSF pleocytosis is noted, other diseases associated with Guillain-
Barré syndrome eg, HIV infection, Lyme disease, malignancy, and
sarcoidosis should be considered
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HUGHES FUNCTIONAL SCALE FOR SGB
HUGHES
FUNCTIONAL KETERANGAN
GRADING SCALE
Grade I Gejala dan tanda minimal; pasien dapat
berlari
Grade 2 Dapat berjalan 5 meter tanpa bantuan
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Treatment
Immunomodulating treatment
The first therapy proven to benefit patients with
Guillain-Barré syndrome is plasmapheresis
(Plasma exchange)
– This procedure mechanically removes humoral factors.
IVIg (intravenous immunoglobulin)
– Dosage : 0.4 g/kg/day for 5 days consecutively
Corticosteroid : no benefit
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Bagaimana pengobatan SGB?
Plasma exchange/plasmapharesis: mengeluarkan
total 200-250 ml/kg BB dalam 4-6 kali pada hari
yang berbeda
Imunoglobulin intra vena (Gamaras@)
Dosis : 0.4 g/kgBB/hari selama 5 hari berturut-turut.
EBM: median waktu perbaikan 27 hari
Corticosteroid: tidak memberi manfaat jangka
panjang
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Treatment
Indication for ICU monitoring
– Rapidly progression (<7 days)
– Inability to raise their head against gravity
– Bulbar dysfunction
– Bilateral facial weakness
– Significant autonomic dysfunction
– Obvious aspiration
– Declining pulmonary function capacity may indicate the
need for mechanical ventilation
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Plasma Exchange
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Prognosis
Self limiting
Improvement expected to begin by 4 weeks
About ¾ recover completely
20% remain with mild deficits (distal numbness or foot-drop,
imbalance, or sensory loss )
5% die - Respiratory failure - Autonomic instability
Chronic CIDP (chronic inflamatory demyelinating
progressive)
Factors associated with a poorer outcome include – Older
age(>50yrs) – Severe, rapidly progressive disease (<1wk) –
Prolonged mechanical ventilation (>1 month) – Persistent,
severely abnormal findings on electromyography
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