Guillain Barre Syndrome

dr. Rohmania Setiarini

It has an annual incidence of 0.6 to 2.4 cases per
100,000 population and occurs at all ages and in
both sexes
With the marked decline in the incidence of polio,
Guillain-Barr syndrome is now the most
common cause of acute flaccid paralysis in healthy
people

Guillain-Barr syndrome:
is a rare immune mediated neuropathy characterized
by progressive muscle weakness
PATHOGENESIS
Peripheral nerve demyelination in Guillain-Barr
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

Clinical feature
Two-thirds of patients develop the neurologic symptoms 2-
4 weeks after what appears to be a benign respiratory or
gastrointestinal infection
The initial symptoms are fine paresthesias in the toes and
fingertips, followed by lower extremity weakness that may
ascend over hours to days to involve the arms, cranial
nerves, and in severe cases the muscles of respiration
Clinical feature
Weakness in lower extremities progresses
over hours to weeks and peaks in 14 days.
Muscles of the distal extremities are severely
affected.
Common sx include: hypotonia (reduced
muscle tone) and areflexia (lack of reflexes).
Autonomic disturbances
Physical Examination
Symmetric limb weakness with diminished or absent
reflexes
Minimal loss of sensation despite paresthesias
Signs of autonomic dysfunction are present in 50 percent
of patients, including
Cardiac dysrhythmias (asystole, bradycardia, sinus
tachycardia, and atrial/ventricular tachyarrhythmias)
Orthostatic hypotension
Transient or persistent hypertension
Paralytic ileus
Bladder dysfunction
Abnormal sweating
Diagnose
Based on pt hx and clinical manifestations.
Lumbar puncture for CSF is normal or low
protein. After 7 days protein is high.
Electromyogram (EMG) test shows that
nerve conduction is slower.
Electrocardiogram (ECG) test shows heart
irregularities.
Nerve biopsy can be done examining the
damaged nerve or axon.
Prognosis
The recovery ranges from 3 w to 3 y.
After 3 y, 30% of the pts will have residual
weakness. The mortality rate is 3% from
GBS.
Treatment
The main modalities of therapy for Guillain-Barr
syndrome include
Plasmapheresis
Administration of intravenous immune globulin
AANs
Class of evidence for therapy
Class I. High quality randomized controlled trials
(RCTs)
Class II. Prospective matched group cohort studies or
RCTs lacking adequate randomization
concealment or blinding, or potentially liable to
attrition or outcome ascertainment bias
Class
III.
Other studies such as natural history studies
Class
IV.
Uncontrolled studies, case series, or expert
opinion
AANs
Recommendation Levels
Level
A
Established as effective, ineffective or harmful,
or as useful/predictive or not useful/predictive
Level
B
Probably effective, ineffective or harmful, or as
useful/predictive or not useful/predictive
Level
C
Possibly effective, ineffective or harmful, or as
useful/predictive or not useful/predictive
Level
U
Data inadequate or conflicting; Treatment, test,
or predictor unproven
Conclusions (PE)

Plasma exchange hastens recovery in non-ambulant
patients with GBS who present within four weeks from the
onset of neuropathic symptoms (Class II evidence).

Plasma exchange also hastens recovery in ambulant
patients who present within two weeks but the evidence is
limited to one trial (Class II evidence).

The effects of plasma exchange and IVIg are equivalent in
patients requiring aid to walk(Class I evidence).

Treatment with CSF filtration has not been adequately
tested (Limited Class II evidence).


American Academy of Neurology
Recommendations

PE is recommended in non-ambulant patients
within four weeks from onset (Level A, Class II
evidence).

PE is recommended for ambulant patients within two
weeks from onset (Level B, limited Class II
evidence).


American Academy of Neurology


Iv immune globuline
Conclusions
Intravenous immunoglobulin has not been
adequately compared with placebo (limited Class II
evidence).
Such comparison is not now needed because, when
started within two weeks from the onset, IVIg has
equivalent efficacy to PE in hastening recovery from
patients with GBS who require aid to walk (Class I
evidence).
Multiple complications were significantly less
frequent with IVIg than with PE (Class I evidence).
There is no evidence concerning the relative efficacy
of PE and IVIg in patients with axonal forms of GBS.
American Academy of Neurology


Recommendations

IVIg is recommended for patients with GBS who
require aid to walk within two (Level A
recommendation) or four weeks from the onset of
neuropathic symptoms (Level B recommendation
derived from Class II evidence concerning PE
started within the first four weeks).

The effects of IVIg and plasma exchange are
equivalent. (Level B recommendation Class I
evidence concerning the comparisons between PE
and IVIg started within the first two weeks).

American Academy of Neurology


Analysis of the evidence
Combination treatments
One Class I trial showed that PE followed by IVIg
showed no significant benefit compared with PE
alone in any measured outcome.
Conclusions
Sequential treatment with PE followed by IVIg does
not have a superior effect to either treatment given
alone (Class I evidence).

Sequential treatment with immunoabsorption
followed by IVIg has not been adequately tested
(Limited Class IV evidence).


American Academy of Neurology


Recommendations

Sequential treatment with PE followed by IVIg is not
recommended (Level A recommendation, Class I
evidence).

Immunoabsorption followed by IVIg is not
recommended (Level U recommendation, Class IV
evidence).


American Academy of Neurology

Analysis of the evidence
Immunoabsorption
An alternative technique to PE, which removes
immunoglobulins.
Has the advantage of not requiring the use of a
human blood product as a replacement fluid.
In a prospective trial there were no differences in
outcome between 11 patients treated with PE and
13 treated with immunoabsorption
There is only limited Class IV evidence from a
single small non-randomized, unblinded study.


Conclusion
The evidence is insufficient to recommend the use of
immunoabsorption (Level U recommendation, Class IV
evidence).


American Academy of Neurology

Recommendation
A 17-year-old male developed flu-like symptoms, severe diarrhoea and
abdominal pain 4 days after attending a dinner party at which he had eaten a
chicken. Three other people who had attended the same party developed
gastrointestinal symptoms. These symptoms settled within a few days. Stool
cultures taken from all four individuals grew Campylobacter jejuni. About 10
days after the onset of diarrhoea, he developed diffuse aching around his
shoulders and buttocks and pins and needles in his hands and feet. Over the
next week the sensory changes worsened and spread to involve his arms and
legs. His limbs became progressively weaker and 8 days after the onset of
neurological symptoms he could not hold a cup or stand unaided. He was
admitted to hospital and found to have severe symmetrical distal limb
weakness and glove and stocking sensory loss to the elbows and knees.
Nerve conduction studies showed evidence of a mixed motor and sensory
neuropathy and examination of his cerebrospinal fluid (CSF) showed a very
high total protein level at 4g/l but without any increase in the number of cells
in the CSF. High titres of IgM and IgG antibodies to Campylobacter jejuni
were found in his peripheral blood.
Case