Guillain-Barré syndrome is an acute inflammatory demyelinating polyneuropathy that is the most common cause of acute flaccid paralysis. It has an annual incidence of 0.6 to 4 cases per 100,000 people and often follows a bacterial or viral infection. Campylobacter jejuni infection is the most commonly identified precipitant. Guillain-Barré syndrome results from an autoimmune attack on the peripheral nervous system that causes muscle weakness and loss of reflexes. Treatment involves supportive care along with plasmapheresis or intravenous immunoglobulin to stop the immune response. Most patients recover fully but some are left with permanent neurological deficits.

1. Dr Sajith Sebastian Resident DNB

2.  Guillain-Barré syndrome is an acute
inflammatory demyelinating polyneuropathy
characterized by progressive muscle weakness
and areflexia
 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

3.  Worldwide, the annual incidence is about 0.6–4
occurrences per 100,000 people.
 Men are one and a half times more likely to be affected
than women.
 The incidence increases with age; there are approximately
1 cases per 100,000 people aged below 30 years and about
4 cases per 100,000 in those older than 75 years.
 The incidence of GBS during pregnancy is 1.7 cases per
100,000 of the population.
 Congenital and neonatal Guillain–Barré syndrome have also
been reported

4.  Peripheral nerve demyelization in GBS 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

6.  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

7.  Campylobacter infectionCampylobacter 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
 Campylobacter jejuni infection and Guillain-Barre
syndromeN Engl J Med 1995 Nov 23;333(21):1374-9

8.  Guillain-Barré is the result of a cell-mediated
immune attack on peripheral nerve myelin
proteins.
 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

10.  The main lesions are acute inflammatory
demyelinating neuropathy and, particularly
in patients with Campylobacter-associated
disease
 With the autoimmune attack there is an
influx of macrophages and other immune-
mediated agents that attack myelin, cause
inflammation and destruction, and leave the
axon unable to support nerve conduction.

11.  The Guillain-Barré 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

12.  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

13.  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

14.  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

15.  Nerve conduction studies:
 Findings depend on subtype of GBS.
 – The majority show demyelinating pattern while – some
patients may show evidence of axonal loss with little or
no demyelination.
 Mostly 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

16.  Inflammatory markers :ESR is usually raised and
CRP is sometimes
 Evidence of SIADH or renal dysfunction
 Antiganglioside antibodies
1. Anti-GM1 • It is positive in 25% of pts and is a
with worse outcome
2. Anti-GD1a • AMAN subtype of GBS
3. Anti-GQ1b • Miller- Fisher syndrome


17.  Respiratory function tests: –
 These may show reduced vital capacity, maximal
inspiratory and expiratory pressures.
 Arterial blood gases may indicate progressive respiratory
failure.
 Infection screen : Campylobacter jejuni, Cytomegalovirus,
Epstein-Barr virus, Herpes simplex virus, Mycoplasma
pneumoniae. HIV antibodies
 Radiological: An MRI of the spine may show selective
anterior spinal nerve root enhancement with gadolinium
and will exclude cervical nerve impingement

21.  Acute inflammatory demyelinating
polyradiculo -neuropathy = AIDP
 Acute motor axonal neuropathy=AMAN •
 Acute motor and sensory axonal
neuropathy= AMSAN
 Miller fisher syndrome =MFS
 Acute Panautonomic Neuropathy

23.  Most common form
 Accounts for around 85– 90% of cases.
 The clinical features are of symmetrical
ascending motor weakness with hypo- or
areflexia.
 Severe cases may develop secondary axonal
damage

24.  2/3s have identifiable preceding event
 50% begin with paresthesias followed by
weakness in legs; 10% begin with arm
weakness; rarely begins in face
 Ophthalmoplegia: partial 15%, total 5%
 Autonomic dysfunction in 65%,
 arrhythmias, hypotension, urinary retention
in 10-15%,

25.  Progresses for days to 4 weeks
 15% with severe disability
 Mortality 3-5%
 CSF: protein may be normal early, elevated
in 90% by clinical nadir, cells< 10 in 95%, >50
suggests HIV
 EDX: prolonged F & distal motor latencies,
conduction block 30-40% in routine studies

26.  More common in Japan ,China, amongst young
people and in the summer months.
 It has an association with precedent infection
with Campylobacter jejuni.
 Clinical features are similar to AIDP but tendon
reflexes may be preserved.
 Electrophysiological testing may distinguish from
other variants as selective motor nerve and
axonal involvement is demonstrated.
 In AMAN the pathological process involves
binding of antibodies to ganglioside antigens on
the axon cell membrane, macrophage invasion,
inflammation and axonal damage.

27.  Pathology:
 axonal plasmalemma at nodes of Ranvier
sometimes limited to physiologic dysfunction
c nodal lengthening. May go on to extension
through axonal basal lamina. Most axons
recover s Wallerian degeneration
 Prognosis similair to AIDP
 Mortality <5%
 EDX: reduced CMAPs c normal F & distal
motor latencies and sensory studies.
Fibrillations in 2-3 weeks

28.  variant of GBS in which both motor and sensory
fibres are involved and which can be demonstrated
on electrophysiological studies.
 It is more severe and associated with prolonged or
even partial recovery.
 Commonly preceded by diarrhea esp. c. jejuni
 Abrupt onset of weakness c rapid progression to
quadriplegia & respiratory insufficiency
 Clinical features are similar to AMAN but also involve
sensory symptoms.


29.  The underlying pathological process is
similar to that for AMAN (i.e. antibody
mediated axonal damage).
 Longer recovery, more residual & mortality
10-15%
 EDX: no response in some motor nerves,
decreased amplitude of the CMAPs,
fibrillations on needle study, absent SNAPs

32.  Ataxia
 Areflexia
 Ophthalmoplegia.
 Benign variant and usually not requiring Immuno-
therapy
 25% of patients may develop limb weakness.
 Electrophysiological studies show primarily sensory
conduction failure.
 Antiganglioside antibodies to GQ1b are found in 90%
of patients and are associated with ophthalmoplegia .
 There have been limited pathological studies in MFS
but demyelination of nerve roots has been
demonstrated.

33.  Studies show preferential location of anti-GQ1b
to cerebellar molecular layer & Cranial Nerves
3,4 & 6
 May act at N-M junction depleting acetylcholine
from nerve terminals

34.  Manifests over 1-2 weeks but may be of
subacute onset
 Frequent preceding infection
 DTRs lost in 1/3, distal sensory changes 1/4
 Albumino cytologic dissociation
 EDX: NCVs usually normal
 Recovery is gradual and incomplete

36.  The main modalities of therapy for Guillain-
Barré syndrome include
 Plasmapheresis and
 Administration of intravenous immune globulin
 Supportive care and monitoring.

38.  IV IG-within 2 or possibly 4 weeks of the onset
of neuropathic symptoms;
 Corticosteroids are not recommended for the
management of GBS.
 sequential treatment with PE followed by IV IG
or immunoabsorption followed IV IG is not
recommended for patients with GBS
 PE and IV IG are treatment options for children
with severe GBS

40.  Supportive Care
• Airway
• respiratory
• Cardiovascular
• Gastrointestinal
• Neurological
• Psychological Rehabilitation

41.  ARDS, sepsis, pulmonary emboli, and
unexplained cardiac arrest.
 Other Factors associated with a poorer
outcome include
 Older age
 Severe, rapidly progressive disease
 Prolonged mechanical ventilation (>1 month)
 Persistent, severely abnormal findings on
electromyography

42.  The majority of patients with GBS either
recover completely or are left with only
minor deficits eg, distal numbness or foot-
drop
 However, 5 to 10 percent of patients will
suffer permanent disabling weakness,
imbalance, or sensory loss
 3 to 8 percent of patients die despite
intensive care
 There may be a recurrence in 2–5% of cases.
 The mortality from GBS ranges from 2–12%.

43.  Thank You