GUILLAIN BARRE

SYNDROME
Bagian Ilmu Penyakit Saraf
RS Bayukarta
Karawang

INFLAMMATORYDEMYELINATING DISORDERS
CLASSIFIED BY COURSE

ACUTE INFLAMMATORY-DEMYELINATING
POLYRADICULONEUROPATHY (AIDP)

CHRONIC INFLAMMATORY-DEMYELINATING
POLYRADICULONEUROPATHY (CIDP)

GUILLAIN BARRE SYNDROME

SUBDIVIDED INTO SUBGROUPS OF CASES :

Variants with rapid progressive phase

ACUTE INFLAMMATORY DEMYELINATING POLYRADICULOPATHY

(AIDP)

80% of GBS patients get this form

ACUTE MOTOR SENSORY AXONAL NEUROPATHY (AMSAN)

a serious axonal form of GBS that attacks motor and sensory

nerves
ACUTE MOTOR AXONAL NEUROPATHY (AMAN)

a particularly severe form; rural areas of China,children and

young adults during summer months
MILLER-FISHER SYNDROME (MFS) :

Encephalo-myeloradiculopathy

Acute Disseminated

A Very rare form of GBS

Affects about 5% of GBS patients
Causes descending paralysis
Loss of tendon reflexes/coordination
Difficulty in walking and standing
Experience tingling and numbness,
dizziness and nausea
Blurred or double vision,facial sagging

Chronic Inflammatory
Demyelinating Polyneuropathy
(CIDP)

Variants with slow progressive phase

Known as chronic GBS or Chronic
Relapsing Polyneuropathy
Much less common than GBS
Evolves over months or years
Relapses occur more frequently
MMN (Multifocal Motor Neuropathy)
MMSD (Multifocal Motor Sensory
Demyelinating Neuropathy)

GUILLAIN BARRE SYNDROME

INCIDENCE

0.6 1.9 CASES PER 100,000 PEOPLE

OCCCUR AT ANY AGE
INCREASES GRADUALLY WITH AGE
THE SEXES ARE EQUALLY AFFECTED
PRECIPITATED BY VACCINATIONS,
GENERAL SURGERY, PREGNANCY,
AND VIRAL INFECTION

CHARACTERISTICS OF GBS
ACUTE ONSET OF PERIPHERAL AND CRANIAL NERVE
DYSFUNCTION
VIRAL UPPER RESPIRATORY OR GI INFECTION,
IMMUNIZATION, OR SURGERY OFTEN PRECEDES
NEUROLOGIC SYMPTOMS BY 5 DAYS TO 3 WEEKS
RAPIDLY PROGRESSIVE SYMMETRIC WEAKNESS
LOSS OF TENDON REFLEXES, FACIAL DIPLEGIA
BULBAR AND RESPIRATORY PARESIS
WORSENS FOR SEVERAL DAYS TO 3 WEEKS
GRADUALLY IMPROVES TO NORMAL OR NEARLY
NORMAL FUNCTION

GUILLAIN BARRE SYNDROME

Pathogenesis
A popular theory suggest that
the organism (virus or bacteria)
responsible for the preceding
infection somehow confuses the
the immune system, perhaps by
mimicking the characteristics of
the nerve cells (molecular
mimicry), making it less
discriminating about what cells it
attacks
Ann Neurol 1995

GUILLAIN BARRE SYNDROME

Pathogenesis
Another suggest that the

organism perhaps changes

the characteristics of the
nerve cells, causing the
immune system to see them
as foreign cells

GUILLAIN BARRE SYNDROME

Pathogenesis
Special proteins: the antibodies or

immunoglobulins are produced by the

immune system as a reaction to the
presence of antigens; in GBS patients
are somehow produced against myelin.
These antibodies circulate in the
blood, and eventually find myelin,
attack and destroy it with the help of
white blood cells, producing
inflammation in the nerves.

GUILLAIN BARRE SYNDROME

Pathogenesis
The inflamed cells in turn secrete

chemicals that affect the Schwann

cells (which produced the fatty
materials required to produce myelin)
The Schwann cells reduce myelin
production, and some of them may
even die, further reducing myelin
production, while at the same time
the existing myelin is destroyed by
the antibodies

GUILLAIN BARRE SYNDROME

Pathogenesis
Tumor necrosis factor-alpha : a
cytokine that has wellrecognized toxic effects on
myelin, may be important in the
pathogenesis of peripheral
demyelination in GBS
Ann Neurol 1993

GUILLAIN BARRE SYNDROME

Pathogenesis
Recently, Campylobacter Jejuni
was claimed to be a predominant
precipitating agent that may also
trigger a hunoral immune
response to glycoconjugates of
peripheral myelin in GBS
Related with axonal type and
poor outcome
Ann Neurol 1993
N Engl J Med 1995

GUILLAIN BARRE SYNDROME

Pathogenesis
A form of GBS occurs after respiratory
infection by Haemophilus Influenzae in
the Japanese population
A particular strain of non-typable
Haemophilus influenzae has a
ganglioside GM1-like structure
Elicits axonal GBS
Brain 2000

PATHOLOGIC FEATURES AND

PROGNOSIS

Segmental demyelination (the

predominant structural change) :

rapidly reversible once repair is
initiated.

Axonal interruption which

occurs distally :
the nerve cell body will survive and
slow regeneration with eventual
recovery will ensue.

PATHOLOGIC FEATURES AND

PROGNOSIS

Axonal interruption which

occurs proximally: the nerve
cell body may die.
But if a nerve remain nonfunctional for a sufficiently long
time, recovery may no longer
possible.

PATHOLOGIC FEATURES AND

PROGNOSIS
Relative proportions of segmental
demyelination and axonal interruption with
muscle denervation determine recovery:
* complete and rapid

* initially rapid but incomplete

and followed by gradual further
improvement over many months/years
* incomplete permanently

GUILLAIN BARRE SYNDROME

SYMPTOMS AND SIGNS
SYMMETRIC WEAKNESS OF THE LIMBS
OFTEN ACCOMPANIED BY PARESTHESIA
OCCASIONALLY, FACIAL, EXTRAOCULAR,OR
OROPHARYNGEAL MUSCLES MAY BE THE FIRST
TO BE AFFECTED
SOME PATIENTS REQUIRE MECHANICAL SUPPORT
OF VENTILATION
HYPORELEXIA OR REFLEXIA IS PRESENT
DEGREE OF SENSORY IMPAIRMENT IS VARIABLE
EVIDENCE OF AUTONOMIC DYSFUNCTION

GUILLAIN BARRE SYNDROME

Course of disease

Progressive phase: last typically 2-3 weeks,

measured from the observation of the first symptom until

no further deterioration occurs.

Plateau phase: neither worsening nor

improvements occurs during unpredictable span
of time (a few days-several months).
Recovery phase: spontaneous improvement
and recovery which is individual, achieved in a few
weeks or after several years

GUILLAIN BARRE SYNDROME

LABORATORY DATA

ELEVATED CSF PROTEIN

CSF NORMAL CELL COUNT
(cyto-albumine dissociation)
THE OCCURRENCE OF
ANTECEDENT VIRAL DISEASES
MAY BE DOCUMENTED BY
SEROLOGIC STUDIES

GUILLAIN BARRE SYNDROME

Diagnosis
Clinical examinations of the
symptoms and their distribution:

symmetrical symptoms,increasing
weakness, signs of preceding infection

History: contact with poisons,alcohol

consumption,recent infections,
diabetes,family history of nerve
disease

Course of the disease

GUILLAIN BARRE SYNDROME

Diagnosis
FURTHER EXAMINATIONS

LABORATORY TESTS: blood and

urine tests, stool test, x-rays, scans,
lumbal puncture
ELECTRODIAGNOSTIC STUDIES:
nerve conduction velocity test,
EMG, ECG

GUILLAIN BARRE SYNDROME

Treatment and care of
patients
Treatment begins as soon as the diagnosis is established

Symptomatic : to reduce symptoms

Immunotherapy : to shorten the
duration of the disease
Physiotherapy and hydrotherapy : to
maintain the bodys muscles and to
reduce stiffness and discomfort of the
extremities
Psychotherapy
Ventilator treatment and
tracheostomy

GUILLAIN BARRE SYNDROME

Treatment and care of patients
Plasmapheresis or Plasma Exchange
(PE) : a mechanical process that
involves the exchange of plasma and
removal of disease-causing
antibodies from the patients blood
Intravenous Immunoglobulin (IVIg) :
consists of the slow injection of
high doses of donor antibodies
(immun-globulins), into the patients
blood

GUILLAIN BARRE SYNDROME

Treatment and care of patients
Plasmapheresis or Plasma Exchange (PE) :
a mechanical process that involves the
exchange of plasma and removal of diseasecausing antibodies from the patients blood
Intravenous Immunoglobulin (IVIg) :
consists of the slow injection of high doses
of donor antibodies (immun-globulins), into
the patients blood
* Immunadsorption (Imad) : resembles PE,
but only the immunoglobulins are removed

GUILLAIN BARRE SYNDROME

Treatment and care of patients
Giving the high doses of other antibodies
causes the patients own destructive
antibodies to disappear into the crowd.
Some of the donor antibodies inactivate
these destructive antibodies, and the
disease is slowed down. The activity of the
white blood corpuscles that produce the
undesirable antibodies is also slowed down.

GUILLAIN BARRE SYNDROME

Recovery
Making a prognosis about recovery is
impossible
Recovery begins as suddenly as when gbs
symptoms appear
The symptoms disappear gradually, but may
take weeks, months or years
The course of the disease varies for each
patient
Recovery takes 3-6 months for most
patients
About two thirds of them recover
completely

GUILLAIN BARRE SYNDROME

Recovery
DEATH : UP TO 5% OF THE CASES,
DUE TO CARDIOVASCULAR OR
RESPIRATORY COMPLICATIONS
OF THE REST, 70% MAKE AN
EXCELLENT RECOVERY WITH NO
PERMANENT DAMAGE, EVEN AFTER A
SEVERE ATTACK
ABOUT 20% ARE DISABLED
ABOUT 10% ARE SEVERELY
DISABLED

GUILLAIN BARRE SYNDROME

Prognosis
Influence by

The onset of recovery

Age
The intensity of infection phase
Need for a ventilator
Major loss of coordination
Degree of paralysis
Preceding diarrhoea
Signs of axonal damage
Duration of the treatment