MOHD SAQUIB KHAN
M.Sc 3rd SEMESTER
DEPARTMENT OF BIOCHEMISTRY &
o Popularly known as “French polio” is an acute inflammatory
demyelinating polyneuropathy characterized by progressive
muscle weakness and areflexia.
o All forms of Guillain–Barré syndrome are autoimmune disease,
due to an immune response to foreign antigens.
o 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.
o With the marked decline in the incidence of polio, GuillainBarré syndrome is now the most common cause of acute
flaccid paralysis in healthy people.
o ACUTE INFLAMMATORY
DEMYELINATING POLYNEUROPATHY(AIDP)- autoimmune
response directed against Schwann cell membranes.
o MILLER FISHER SYNDROME (MFS)- Anti-GQ1b antibodies
are present in 90% of cases.
o ACUTE MOTOR AXONAL NEUROPATHY (AMAN) also
known as Chinese paralytic syndrome- Anti-GD3 antibodies
are found more frequently in AMAN.
o ACUTE PANAUTONOMIC NEUROPATHY- associated with a
high mortality rate, owing to cardiovascular involvement, and
o Campylobacter infection is the most commonly identified
precipitant of Guillain-Barré syndrome.
o 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.
o Seventy percent of those infected with C. jejuni reported
a diarrheal illness within 12 weeks before the onset of
the neurologic illness.
o The main lesions are acute inflammatory demyelinating
neuropathy and, particularly in patients with
Campylobacter-associated disease, acute axonal
o 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
o However, mechanisms other than molecular mimicry
may be associated with the production of antibodies to
o Anti-GD3-Anti-GD3 antibodies have been found in
association with specific forms of Guillain-Barré
o In vivo studies of isolated anti-GM1 and GD3
antibodies indicate the antibodies can interfere with
motor neuron function.
o Anti-GD1a antibodies were highly associated acute
motor axonal neuropathy while high titers of anti-GM1
were more frequent indicating that GD1a possibly
targets the axolemma and nodes of ranvier. .
o Anti-GM1-Levels of anti-GM1 are elevated in
patients with various forms of dementia &
correlate with more severe Guillain-Barré
o Titers to GM1 in other diseases (rheumatoid
arthritis, primary Sjögren's syndrome and
systemic lupus erythematosus) was also
o The autoimmune role of anti-GM1 is still unclear.
o Anti-GQ1-Anti-GQ1b are found in MillerFisher syndrome.
o Studies of these antibodies reveal large
disruption of the Schwann cells.
o Anti-GQ1b IgG levels were elevated in
patients with ophthalmoplegia in GuillainBarré syndrome.
o In Guillain-Barré syndrome, the myelin sheath
surrounding the axon is lost.
o Demyelination is a common response of neural
tissue to many agents and conditions, including
physical trauma, hypoxemia, toxic chemicals,
vascular insufficiency, and immunological
o Loss of the myelin sheath in Guillain-Barré
syndrome makes nerve impulse transmission is
o The syndrome may develop rapidly over the course
of hours or days, or may take up to 3 to 4 weeks to
o Most patients demonstrate the greatest weakness
in the first weeks of the disorder.
o Patients are at their weakest point by the third
week of the illness.
o In the beginning, a flaccid, ascending paralysis
o The patient may first notice weakness in the
lower extremities that may quickly extend to
include weakness and abnormal sensations in
o Deep tendon reflexes are usually lost, even in
the earliest stages.
o The trunk and cranial nerves may become
o Respiratory muscles can become affected,
resulting in respiratory compromise.
o The history of the onset of symptoms can be
revealing because symptoms of Guillain-Barré
syndrome usually begin with weakness or
paresthesias of the lower extremities and
ascend in a symmetrical pattern.
o A lumbar puncture may be performed and reveal
o Also, nerve conduction studies record impulse
transmission along the nerve fiber.
o The main modalities of therapy for Guillain-Barré
– Administration of intravenous immune globulin
o The first therapy proven to benefit patients with
Guillain-Barré syndrome is plasmapheresis.
o This procedure mechanically removes humoral
o Plasma exchange is recommended for patients
– Are unable to walk unaided
– Demonstrate worsening vital capacities
– Require mechanical ventilation
– Have significant bulbar weakness
o Intravenous immunoglobulin (IVIG) is also useful
in managing Guillain-Barré syndrome.
o Davids, H. "Guillain-Barre Syndrome".
Medscape Reference. Retrieved 3 Jan 2012.
o Jump up Mori, M; Kuwabara, S; Fukutake, T;
Hattori, T (2002). "Plasmapheresis and Miller
Fisher syndrome: analysis of 50 consecutive
cases". Journal of neurology, neurosurgery, and
psychiatry 72 (5).
o Thomas J.Kindt,Barabara A.Osborne,Richard
A.Goldsby.Kuby Immunology 6th edition.
developed GuillainBarré syndrome in
1983. Griffith is seen
here receiving an
award at the White
House in 2005.