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Ken Wirastuti
Bagian IP. Saraf
FK. UNISSULA - Semarang
History
History of GBS
1830s
1859
- Landry -- detailed description
paralysis)
(Landrys
1916
- Guillain, Barr and Strohl - disease
accompanied by hyperalbuminosis in CSF
1936
1950s
- Electromyography
1969
- Asbury - inflammation is quintessential
feature of GBS
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Synonim:
polineuritis post infectiosa
acute inflamatory polyneuropathy
acute autoimmune neuropathy
acute idiopathic polyradiculoneuritis
Landry Gullain Barre Syndrome
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Epidemiology
Annual Incidence: 0.4-4.0 (median 1.3) : 100,000
Age and Sex: Occur at all ages (8mo - 81yr)
2 peak
Late adolescence and young adulthood
Elderly
Men : women = 1.25 : 1
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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-Barr 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 GuillainBarr 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 myelinproducing Schwann cells
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Pathogenesis
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History
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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
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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%)
Facial diplegia
Areflexia
Muscle wasting (after 2 wks.)
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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
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Autonomic dysfunctions
Fluctuation of heart rate and
blood pressure
Hypersalivation
Excessive/abnormal sweating
Paralytic ileus
Urinary retention
Constipation
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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
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Treatment
Supportive Treatment
25-30% require intubation and mechanical
ventilation
Continuous monitoring of blood pressure and
heart rate
Immunomodulating treatment
Plasma exchange
IVIg
Corticosteroid : no benefit
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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
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Immunoglobulin
Plasma exchange and IVIg have equivalent
efficacy
Advantages of lower risk and ease of application
IVIg is more efficacious than plasma exchange
in patients with axonal GBS
Dosage : 0.4 g/kg/day for 5 days consecutively
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Prognosis
Self limiting
Improvement expected to begin by 4 weeks
About recover completely
20% remain with mild deficits (distal numbness or footdrop, 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 Severe, rapidly progressive disease Prolonged
mechanical ventilation (>1 month) Persistent, severely
abnormal findings on electromyography
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