3. 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-Barré syndrome is now the most
common cause of acute flaccid paralysis in
healthy people
is an acute inflammatory demyelinating
polyneuropathy characterized by progressive muscle
weakness and areflexia
4. Guillain-Barre’ Syndrome
Post-infectious polyneuropathy; ascending
polyneuropathic paralysis
An acute, rapidly progressing and potentially fatal
form of polyneuritis
7. PATHOGENESIS
Peripheral nerve demyelination in
Guillain-Barré 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
8.
9. Guillain-Barre’
T-cell sensitization occurs which causes
loss of myelin which disrupts nerve
impulses
Loss of myelin, edema and
inflammation of the affected nerves,
causes a loss of neurotransmission to
the periphery.
85% of patients recover with supportive
care.
10.
11. Guillain-Barré 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
12. Campylobacter infection
is the most commonly identified precipitant of
Guillain-Barré 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
13.
14.
15. Clinical Manifestations
Usually develop 1 to 3 weeks after URI or GI
infection
Weakness of lower extremities
(symmetrically)
Parathesia (numbness and tingling), followed
by paralysis
Hypotonia and areflexia (absence of reflexes)
Pain in the form of muscles cramps or
hyperesthesias (worse at night).
16. 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
17. 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
18. Clinical manifestations
Autonomic nervous system dysfunction
results from alterations in sympathetic
and parasympathetic nervous systems.
Results in respiratory muscle paralysis,
hypotension, hypertension, bradycardia,
heart block, asystole.
Involvement of lower brainstem leads to
facial and eye weakness
19.
20. 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
21. 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-Barré syndrome
unlikely
22. 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-Barré syndrome eg,
HIV infection, Lyme disease, malignancy, and
sarcoidosis should be considered
23. Therapeutic management
Ventilator support!
Plasmapheresis used within the first 2 weeks
of onset. If treated within the first 2 weeks,
LOS of morbidity is reduced. After three
weeks, plasmapharesis no benefit.
IV immunoglobin
Nutritional support (TF, TPN, Diet)
24.
25.
26. Variants
Acute inflammatory
demyelinating polyneuropathy (AID
P) is the most common form of GBS,
and the term is often used
synonymously with GBS.
It is caused by an autoimmune response
directed against Schwann
cell membranes.
27. Miller Fisher syndrome (MFS) is a
rare variant of GBS. Accounting for
about 5% of GBS cases, it manifests as a
descending paralysis, proceeding in the
reverse order of the more common
form of GBS.
28. Acute motor axonal
neuropathy (AMAN), also known
as Chinese paralytic syndrome, attacks
motor nodes of Ranvier and is prevalent
in China and Mexico.
It is probably due to an auto-immune
response directed against the
axoplasm of peripheral nerves.
29. Acute motor sensory axonal
neuropathy (AMSAN) is similar to
AMAN, but also affects sensory nerves
with severe axonal damage.
Acute panautonomic neuropathy is
the rarest variant of GBS, sometimes
accompanied by encephalopathy.
30. Bickerstaff's brainstem
encephalitis (BBE), a further variant of
Guillain–Barré syndrome, is
characterized by acute onset
of ophthalmoplegia, ataxia, disturbance
of consciousness, hyperreflexia
or Babinski's sign.
