Guillain-Barre Syndrome

Guillain-Barre Syndrome
Ken Wirastuti, M Kes, Sp.S, KIC

Bagian IP. Saraf
FK. UNISSULA - Semarang
History
1830s - 1st clinical descriptions
1859 - Landry -- detailed description (Landry’s paralysis)
1916 - Guillain, Barré and Strohl - disease accompanied by
“hyperalbuminosis” in CSF
1936 - Guillain - cardinal clinical features
1950s - Electromyography
1969 - Asbury - inflammation is quintessential feature of GBS
Page  2
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.
Page  3
Synonim:
 Polineuritis post infectiosa

 Acute inflamatory polyneuropathy
 Acute autoimmune neuropathy
 Acute idiopathic polyradiculoneuritis
 Landry Gullain Barre Syndrome
Page  4
Epidemiology
 Annual Incidence: 0.4-4.0 per 100,000 (median 1.3)

 Age and Sex: Occur at all ages (8 mo – 81 yr)
 2 peak
– Late adolescence and young adulthood (1 per
100.000 in < 30 yrs of age)
– Elderly (4 per 100.000 in > 75 yrs of age)
 Men : women = 1.25-1.5 : 1
Page  5
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
Page  6
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.
Page  7
Pathogenesis
Page  8
History
 Often follows minor infection

 Upper Rerpiratory Tractus Infection
 Diarrhoea ( Campylobacter)
 Progressive symmetrical limb weakness
 Usually affects legs first and ascends
 Proximal > Distal weakness
 Frequent sensory complaints
Page  9
 Progresses over no more than 4 weeks
Page  10
Clinical Features
Paresthesias and numbness are frequent and

early symptoms
Major clinical manifestation is muscle
weakness
Maximum deficit develop over days and nadir
in 2 wks. (no progression after 4 wks.)
followed by a plateau phase and gradual
recovery
Page  11
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.
Page  12
Sensory symptoms
Pain and an aching discomfort in the muscles,

mainly those of the hips, thighs and backs
Numbness, paresthesia
(glove & stocking patern)
Lost of joint position sense,
vibration, touch and
pain distally
Ataxia
Page  13
Autonomic dysfunctions
Fluctuation of heart rate and blood

pressure
Orthostatic hypotension
Hypersalivation
Excessive/abnormal sweating
Paralytic ileus
Urinary retention
Constipation
Page  14
Monitoring
Neurological examination
Complaints of dyspnea
Cardiac monitor
Blood pressure
Vital capacity
Page  15
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
Page  17
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
Page  18
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
Grade 3 Dapat berjalan 5 meter dengan alat

bantu
Grade 4 Duduk atau berbaring
Grade 5 Perlu ventilasi

Page  19
Komplikasi
Infeksi pernafasan
Sepsis
Gangguan irama jantung
Hipertensi/hipotensi
Aspirasi
Kelumpuhan permanen
Thrombosis vena dalam
Dekubitus
Page  20
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
Page  21
Page  22
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
Page  23
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
Page  24
Plasma Exchange
 PE recommended for patients

– Unable to walk unaided
– Worsening vital capacities
– Require mechanical ventilation
– Significant bulbar weakness
 Removes a total of 200-250ml/kg in
4-6 treatments on alternate days
Page  25
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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Guillain-Barre Syndrome

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Guillain-Barre Syndrome

Ken Wirastuti, M Kes, Sp.S, KIC

 Polineuritis post infectiosa

 Annual Incidence: 0.4-4.0 per 100,000 (median 1.3)

 Often follows minor infection

Paresthesias and numbness are frequent and

Pain and an aching discomfort in the muscles,

Fluctuation of heart rate and blood

Grade 3 Dapat berjalan 5 meter dengan alat

Grade 5 Perlu ventilasi

 PE recommended for patients

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