Guillain Barre Syndrome

Guillain Barre Syndrome ( GBS ) a group of

immune

mediated

peripheral

nerves

system

disorder
The most frequent cause of acute generalized
paralysis
Since the virtual elimination of poliomyelitis, GBS
has become the leading cause of acute flacid
paralysis in western country and development
country

Epidemiology
Udaya 2000 : incidence GBS 1 3 / 100.000 population in
Europe, USA and Australia. Peak in young adult and
eldery
Allan, 1994 : incidence 1.7 cases per 100.000, and 35
patients with respiratory failure, mortality 1 5 %
RSHS 2000 : 24 patient 4 patient died with respiratory
failure
2001 : 21 patients : 3 patient died, 1 patient in ICU

Clinical features of Guillain Barre Syndrome

Motor dysfunction
Symmetrical limb weakness : proximal, distal, or global
Neck muscle weakness
Respiratory muscle weakness
Cranial nerve palsies : III-VII, IX-XII
Areflexia
Wasting of limb muscles

 Sensory dysfunction
Pain
Numbness, paraesthesiae
Loss of joint position sense, vibration, touch and pain
distally
Ataxia

 Autonomic dysfunction
Sinus tachycardia and bradycardia
Other cardiac arrhytmias ( both tachy and brady )
Hypertension and postural hypotension
Wide fluctuations of pulse and blood pressure
Hypersalivation
Anhydrosis or excessive sweating
Urinary sphincter disturbances
Constipation
Gastric dysmotility
Abnormal vasomotor tone causing venous pooling and facial
flushing

Guillain Barre Syndrome and variants

Weakness Is Predominant
Acute inflammatory demyelinating Polyradiculoneuropathy
( AIDP )
Acute motor axonal neuropathy ( AMAN )
Acute motor sensory axonal neuropathy ( AMSAN )

Weakness Is Not Predominant

Fisher syndrome
Acute panautonomic neuropathy
Pure sensory neuropathy

Features of Acute Inflammatory Demyelinating

Polyradiculoneuropathy ( AIDP )
CLINICAL
Two-thirds of patients have antecedent infection or another
provocative event
Symptoms begin with paresthesias and pain (50%) followed
by muscles weakness in the legs; about 10 % begin with
arm weakness; rarely weakness begins in the face.
Complete ophthalmoplegia in 3 % - 5 %; partial in 15 %
Autonomic manifestations include labile blood pressure,
cardiac arrhythmias, bladder dysfunction, constipation,
abdominal distension and bolating
Disease progresses for days to 4 weeks. The average time
to onset of recovery is 4 weeks
80 % of patients recovery within 6 months
15 % have severe residual disability
Mortality rate 3 % - 5 %

Features of Acute Motor Sensory Axonal Neuropathy

(AMSAN)
CLINICAL FEATURES
Commonly preceded by diarrhea, especially related to
compylobacter jejuni infection.
Abrupt onset of weakness, with rapid progression to
quadriplegia and often early respiratory insufficiency.
Patients may have facial weakness, ophthalmoparesis, and
autonomic instability
Patients have longer recovery periods with significant
residual deficits than in AIDP
Mortality rate 5 % - 10 %

Features of Acute Motor Axonal Neuropathy ( AMAN )

CLINICAL FEATURES
The condition is often preceded by a history of diarrhea,
especially Campylobacter jejuni
Affects children and young adults in Northern China
primarily, and elsewhere; it occurs sporadically in North
America
Mortality rate 5 %
CEREBROSPINAL FLUID CHANGES
Protein is elevated in most cases after the first week
Spinal fluid cell counts are normal

Features of Fisher Syndrome

CLINICAL FEATURES
Syndrome usually begins with diplopia, followed in
3 4 days by limb and gait ataxia
Complete ophthalmoplegia evolves over several
days
Areflexia is typical
Sensory loss is usually mild in the distal limbs
A mild degree of muscle weakness may be
present
There is an excellent prognosis for full recovery

Features of Fisher Syndrome ( contd )

CEREBROSPINAL FLUID
The protein is usually elevated after 7 10 days of
illness
No significant pleocytosis occurs
ELECTRODIAGNOSTIC STUDIES
Decreased amplitude of sensory nerve action
potentials that return with time is common
Sensory conduction velocities are normal, as are
motor nerve conduction studies

Features of Acute Panautonomic Neuropathy

CLINICAL FEATURES
Onset occurs over 1 2 weeks in most patients
but may be subacute ( over 8 weeks )
Manifestations include : lightheadedness,
dizziness, orthostatic hypotension, nausea,
vomiting, diarrhea, constipation, and postprandial
bloating.
Other findings include : heat intolerance,
decreased sweating, blurred vision, dry eyes,
voiding problems, and impotence
Muscle strech reflexes are lost in one third of
patients, and distal sensory loss in one fourth
CEREBROSPINAL FLUID
Elevated protein without pleocytosis occurs in the

Features of Pure Sensory Neuropathy

Clinical features :
Small number of cases GBS
Manifestation include : ataxia, areflexi, sensory
neuropathy, little or no motor involvement.
Severe cases may have of the face and trunk,
have an common

Antecedent Events for Guillain Barre Syndrome

INFECTIONS
Viral
Epstein Barr virus
Cytomegalovirus
Human immunodeficiency virus
Influenza viruses
Coxsackie viruses
Herpes simplex
Hepatitis A and C viruses
Others*
* Isolated reports of various individual viruses or bacteria

INFECTIONS
Bacterial infections
Campylobacter jejuni
Mycoplasma pneumoniae
Escherichia coli
Other*
Parasitic
Malaria
Toxoplasmosis
* Isolated reports of various individual viruses or bacteria

SISTEMIC ILLNESS
Hodgkins disease
Chronic lymphocytic leukemia
Hyperthyroidism
Collagen vascular diseases
Sarcoidosis
Renal disease

OTHER MEDICAL CONDITIONS

Pregnancy
Surgical procedures
Bone marrow transplantation
Immunizations ( e.g.,swine flu )
Envenomization
Drug ingestion

Criteria for admitting GBS patients to ICU

Vital capacity less than 12 ml / kg
Deteriorating vital capacity less than 18 to 20
mL/kg; clinical signs of diaphragmatic fatigue
including tachypnea, diaphoresis, paradoxical
breathing
Poor cough, accumulating secretions, aspiration
pneumonia
Progressive weakness associated with difficulty
swallowing
Major dysautonomic features ( wide blood pressure
and pulse fluctuations : arrhythmias, heart block,
pulmonary edema, profound ileus with risk of
visceral rupture )
Hypotension precipitated by plasma exchange, or
plasma exchange planned in a ventilated or

Autonomic dysfunction
Dysautonomia
Sinus tachycardia
Labile heart rate
Orthostatic hypotension
Sustained hypertension
Paroxysmal hypertension
Vagal spells
Other arrhythmias
Abnormal drug responses
Urinary retention
Urinary incontinence
Impotence ( males )
Constipation
Ileus
Fecal incontinence

No. cases

Percent

62
14
32
5
40
13
8
2
46
4
2
24
15
2

37
8
19
3
24
8
5
1
27
2
2
14
9
1

ICU Complication

Mortality 1 5 %
Tracheostomy pneumoni
Urinary infection
Phlebilitis
Pulmonary emboli
Depression

Treatment
Supportive care remains unequivocally the most
important component of treatment
Essentially all patient should be observed in
hospital for at least 2 3 week
Patient with severe disease especially there
with respiratory insufficien requiring intubation.
And with autonomic instability need closed
observation and manually in an Intensive Care
Unit

 * Prevent the multiple medical

complication as result prolonged

Immobility
* Rehabilitation effort during the acute
phases
* Pain control
* Psychological suport for patient and
families

Outcome and prognosis

Udaya : - 36 % improvement the first week
- 85 % improvement fourth week
Death rate 13 % become 5 % in ICU
Cause of death : - cardiac arest 25 %
- respiratory failure 75 %

Poor Prognostic Features

for Guillain Barre Syndrome
Older age
Rapid onset prior to presentation ( 7 days
)
Ventilator dependency
Inexcitable or reduced amplitude motor
evoked responses
No treatment ( plasma exchange or
intravenous immunoglobulin )
Preceding diarrheal illness

Factor associated with poor outcome

Aetiology
Previous gastrointestinal infection
Cytomegalovirus
Clinical features
Older age
Shorter latency to nadir
Longer time to clinical improvement
Need for mechanical ventilation
Greater disability and disease severity

 Electrophysiology
Absent or reduced CMAP ( mean distal CMAP
amplitude 20 % of the lower limit normal )
Inexcitable nerves
Biochemical markers
Anti-GM1 antibodies
Neurone specific enolase and S-100b proteins
in CSF