GUILLAIN BARRE

SYNDROME

M.WIDIASTUTI SAMEKTO
FAK.KEDOKTERAN UNDIP
 INFLAMMATORY-
DEMYELINATING 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) : Acute Disseminated

Encephalo-myeloradiculopathy
• 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 well-recognized
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 non-functional 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 body’s 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 patient’s 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 disease-
causing antibodies from the patient’s 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 patient’s 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