Guillain-Barr Syndrome

Mohamed Sulaiman Al-Houqani

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 1
A diagnosis was made of the GuillainBarr syndrome
(acute inflammatory polyneuropathy) probably triggered by
Campylobacter jejuni infection. He was treated with high-
dose intravenous immunoglobulin but his condition
deteriorated with respiratory muscle weakness and he
required mechanical ventilation. His condition slowly
improved and he was able to breathe spontaneously after 2
weeks. His strength and sensory symptoms slowly improved
with vigorous physiotherapy but 1 year after the initial illness
he still had significant weakness in his hands and feet.
A previously healthy 15-year-old black adolescent, gravida
1, para 0, at week 10 of gestation had a 10-day history of
progressive weakness and paresthesia of the lower
extremities. There was no history of a preceding infection or
flu-like illness. The patient reported no symptoms in the
upper extremities or shortness of breath. Neurologic
examination revealed bilateral foot drop, with 4/5 strength of
proximal muscles of the lower extremities and normal
strength in the upper extremities and bulbar muscles.
Sensory examination was remarkable for symmetrically
decreased sense of vibration, light touch, and
proprioception in the lower extremities (below the knees).
Deep tendon reflexes were absent in the lower extremities
and only trace reflexes were present in the upper
extremities. Plantar flexor response was present bilaterally.
Case 2
Baseline vital capacity was 2.47 L (87% of predicted value
for patient age). Thyroid function tests and screening for
heavy metals, vitamin B12, and folate yielded normal
results; erythrocyte sedimentation rate was within normal
limits. Serum protein electrophoresis revealed a normal
migration pattern. The patient did not consent to lumbar
puncture. Motor nerve conduction studies revealed
markedly increased distal latency of the peripheral nerves
of upper and lower extremities, as well as unelicitable F
wave of the peroneal nerves, consistent with acute
demyelinating peripheral polyneuropathy. An
antiperipheral nerve antibody panel revealed mild
elevation of GM1 antibodies (12; normal <10), with normal
values for asialo-GM1, GD1a, and GD1b.
Pelvic ultrasonography revealed a normal intrauterine
pregnancy, good correlation between size and gestational
age, and a live and active fetus. After obstetric
consultation, the patient had four sessions of
plasmapheresis, with a calculated plasma-to-crystalloid
exchange rate of 200 to 250 mL. The patient and fetus
tolerated the procedure well. Progression of motor
symptoms was arrested and a mild improvement was
noticed immediately after the third plasma exchange
session. Follow-up spirometries were normal and the
patient did not require assisted ventilation. Follow-up
ultrasonography did not show any signs of fetal distress.
Aggressive physical therapy was begun and the patient
was ambulatory on discharge from the hospital, 9 days
after admission.
The patient received follow-up care at the High Risk
Obstetrical Clinic and had an uneventful course until
gestational week 20, when a decrease in frequency of
fetal movements prompted immediate ultrasonography.
Intrauterine fetal death was detected, and the patient was
readmitted for an induced vaginal delivery. Failure to
deliver the placenta required cervical dilation with
evacuation of uterine contents and curettage.
Pathologic examination of placental tissue revealed CMV
placentitis.

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 an acute inflammatory demyelinating
polyneuropathy characterized by progressive muscle
weakness and areflexia
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
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, and
infection with Campylobacter, Lyme disease, EBV,
CMV, HSV, mycoplasma, and recently acquired
HIV infection
Campylobacter infection
Campylobacter infection is the most commonly
identified precipitant of Guillain-Barr syndrome
A case-control study involving 103 patients with the
disease found that 26% of affected individuals had
evidence of recent C. jejuni infection compared with
2% of household and 1% of age-matched controls
Seventy percent of those infected with C. jejuni
reported a diarrheal illness within 12 weeks before
the onset of the neurologic illness
The main lesions are acute inflammatory
demyelinating neuropathy and, particularly in
patients with Campylobacter-associated
disease, acute axonal degeneration
These changes may be caused by cross-
reacting antibodies to GM1 ganglioside
(present in high concentrations in peripheral
nerve myelin) formed in response to similar
epitopes expressed by the infecting
Campylobacter strain
However, mechanisms other than molecular
mimicry may be associated with the
production of antibodies to GM1 ganglioside
The Guillain-Barr syndrome variant
known as Miller Fisher syndrome, in
which the cranial nerves are affected, is
also associated with Campylobacter
infection
In these patients cross-reacting
antibodies to GQ1b ganglioside, which
is present in cranial nerve myelin, have
been found
CLINICAL FEATURES
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 FEATURES
Early in the course, patients frequently complain of
aching or sciatica-like lower back or leg pain
At some point during their illness, up to 25 percent
of patients require mechanical ventilation
More than 90% of patients reach the nadir of their
function within two to four weeks, with return of
function occurring slowly over weeks to months
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
DIAGNOSTIC STUDIES
Electrophysiologic studies 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
A normal study after several days of symptoms,
makes the diagnosis of Guillain-Barr syndrome
unlikely
DIAGNOSTIC STUDIES
After the first week of symptoms, analysis of
the cerebrospinal fluid (CSF) typically reveals
normal pressures
few cells (typically mononuclear)
an elevated protein conc. (greater than 50 mg/dL)
Early in the course (less than one week),
protein levels may not yet be elevated, but
only rarely do they remain persistently normal
If CSF pleocytosis is noted, other diseases
associated with Guillain-Barr syndrome eg,
HIV infection, Lyme disease, malignancy, and
sarcoidosis should be considered
TREATMENT
The main modalities of therapy for Guillain-Barr
syndrome include
Plasmapheresis and
Administration of intravenous immune
globulin
Plasmapheresis
Plasma exchange is recommended for patients who
Are unable to walk unaided
Demonstrate worsening vital capacities
Require mechanical ventilation
Have significant bulbar weakness
As a result of the cost, risk, and discomfort to the
patient, plasma exchange is generally not used for
ambulatory patients with mild disease or for patients
whose symptoms are no longer progressing after
three weeks
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