The document outlines Guillain-Barré Syndrome (GBS), its history, types, clinical presentation, pathology, diagnosis, and treatment options, particularly focusing on intravenous immunoglobulin (IVIG) therapy. GBS manifests as an acute inflammatory demyelinating polyneuropathy with various subtypes and is often postinfectious in nature. Current evidence supports IVIG and plasma exchange as effective treatments, with guidelines recommending both for hastening recovery, while noting that IVIG is generally safer and easier to administer.

1. GBS AND ROLE OF
IVIG
Clinical Evidence

2. Outline
 GBS – overview
 Brief history
 What ?
 Types of GBS
 Etiology and pathogenesis
 Diagnosis and treatment
 IVIg
 What and how it works ?
 IVIg in GBS
 Current Clinical review of articles
 Recommendations

3. Overview
GBS
Source:
• Van den Berg, B. et al. Guillain–Barré syndrome: pathogenesis, diagnosis, treatment and prognosis; Nat.
Rev. Neurol. advance online publication 15 July 2014
• http://www.uptodate.com/contents/treatment-and-prognosis-of-guillain-barre-syndrome-in-
adults?source=search_result&search=Guillian+barre&selectedTitle=3~150
• Guillain Barre syndrome. Medscape reference 2014

4. Brushing
the
History
 In 1859, Landry published a report on 10
patients with an ascending paralysis.
 Subsequently, in 1916, 3 French physicians
Guillain, Barré and Strohl
 Described 2 French soldiers
 With motor weakness, areflexia, cerebrospinal
fluid (CSF) Albuminocytologic dissociation, and
diminished deep tendon reflexes
 The identified syndrome was later named
Guillain-Barré syndrome.
 Historically, GBS was a single disorder;
however, current practice acknowledges
several variant forms.

5. GBS – What ?
 Guillain-Barré syndrome is an acute
inflammatory demyelinating polyneuropathy
characterized by
 Progressive symmetrical muscle weakness
 & Hyporeflexia / Areflexia
 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

6. GBS – How much common ?
 Rare disease
 Incidence of 0.81–1.89
(median 1.11) per 100,000
person–years
 Less frequent in children
than adults
 Men > Women (Ratio 3:2)
 Incidence increases by
20% for every 10 years
increase in age

7. Common clinical presentations
Types of GBS

8. GBS – Common Types
 Heterogeneous syndrome with several variant
forms
 The major forms are :
 Acute inflammatory demyelinating
polyradiculoneuropathy (AIDP)
 Miller Fisher syndrome (MFS)
 Acute motor axonal neuropathy (AMAN)
 Acute sensorimotor axonal neuropathy (AMSAN)

9. Various types – at glance
GBS subtypes Main clinical
Features
NCS findings Antibodies *
Acute inflammatory
demyelinating
polyneuropathy
(AIDP)
Sensorimotor GBS,
often combined with
cranial nerve deficits
and frequent
autonomic dysfunction
Demyelinating
polyneuropathy
Various‡
Acute motor axonal
neuropathy
(AMAN)
Pure motor GBS;
cranial nerves rarely
affected
Axonal
polyneuropathy,
sensory action
potential normal
GM1a, GM1b
GD1a
GalNAc-GD1a
Acute motor sensory
axonal neuropathy
(AMSAN)
Resembles severe
AMAN, but sensory
fibers are affected,
leading to sensory
deficits
Axonal
polyneuropathy,
sensory action
potential reduced or
absent
GM1, GD1a
Miller Fisher
syndrome
Ataxia,
ophthalmoplegia,
Normal in most
patients; discrete
GQ1b, GT1a

10. GBS – How it presents ?
 Highly variable
 May present with mild difficulty in walking to
complete paralysis of facial, respiratory or bulbar
muscles
 15-30% patients require Mechanical
Ventilation
 20 % are having severe autonomic dysfunction
 Considering this, around half of the patients
require ICU monitoring

11. Common etiology and proposed pathogenetic mechanism
WHY & HOW ?

12. GBS – Why it occurs ?
 GBS is a postinfectious
disorder
 2/3 of patients report
symptoms of a
respiratory or GI tract
infection before the onset
of GBS
 Most common pathogen :
C. jejuni
 Other Pathogens :
Cytomegalovirus (CMV),
Epstein– Barr virus (EBV),
Mycoplasma pneumonia,
Haemophilus influenzae
and influenza A virus

13. Proposed
Pathogenesis
Molecular mimicry of pathogen-borne antigens, leading to generation of cross-
reactive antibodies that also target gangliosides

14. DIAGNOSIS AND TREATMENT

15. How to Diagnose ?
 Clinical Features
 CSF finding:
 Albuminocytologic Dissociation
 CSF protein increases
 Normal CSF WBC count
 Electrophysiological findings – Nerve
conductions studies to differentiate from
demyelinating from axonal subtypes

16. Treatment
 Combating
Respiratory failure
 Cardiovascular
Management
 Bowel & Bladder
care
 Pain control
 Rehabilitation
 Plasmapheresis/Pla
sma Exchange (PE)
 Intravenous
immune globulin
(IVIg)
 Glucocorticoids
(History)
 Interferon beta (only
few cases)
Supportive Care
Disease Modifying
Agents

17. How it works in GBS ?
IVIg (Intravenous Immunoglobulin)

18. IVIg is
therapeutic
preparations
of pooled
polyspecific
IgG obtained
from the
plasma of a
large
number of
healthy
individuals
(>>1000
donors)

19.  Pleotropic immunomodulatory effects
 Don’t know exactly which effect is responsible
for its therapeutic efficacy in GBS
 Possible Mechanism for therapeutic
effects:
 May inhibit Fc-mediated activation of immune
cells
 binding of antiganglioside antibodies to their
neural targets or local complement activation

20.  Commonly used dose in GBS:
 0.4 gm / kg / day
 For 5 days
 Side effects:
 Aseptic meningitis, rash, acute renal failure
(mostly related to sucrose containing products),
and (rarely) hyperviscosity leading to stroke. IgA
deficiency can lead to anaphylaxis

21. Current Clinical Review
IVIg ( Intravenous Immunoglobulin)

22. Cochrane Review – 2014
Objectives :
1. To examine the efficacy of
intravenous Immunoglobulin
(IVIg) in Guillain-Barré
syndrome (GBS)
2. To determine the most
efficacious dose of IVIg
3. To compare the efficacy of
IVIg and plasma exchange
(PE)
4. To compare the efficacy of
IVIg added to PE with PE
alone

23. Selection of studies
Selection Criteria:
 Randomized and quasi-
randomized trials of IVIg
(Adults & children with
GBS of all degrees of
severity) compared
 With no treatment
 Placebo treatment
 PE or other
immunomodulatory
treatments
 Also included trials in
which IVIg was added to
another treatment
Searched :
 The Cochrane
Neuromuscular Disease
Group Specialized
Register (2 December
2013)
 CENTRAL (2013, Issue 12
in The Cochrane Library)
 MEDLINE (January 1966
to November 2013)
 & EMBASE (January 1980
to November 2013).
Total 12 trials were found
eligible for this

24. Key Findings
 536 patients (from 5 trials) comparing IVIg to PE
 Mean difference (MD) of change in a seven-grade disability scale
after four weeks
 No statistical difference between 2 groups
 75 children (from 3 studies):
 IVIg significantly hastens recovery compared with supportive
care
 249 patients (from 1 trial) comparing PE followed by IVIg
with PE alone
 mean grade improvement was 0.2 (95% CI -0.14 to 0.54) more in
the combined treatment group than in the PE alone group

25. Author’s conclusion
 IVIg and PE produced a similar amount of
improvement
 People were more likely to finish a course of
IVIg compared to PE
 IVIg started within two weeks from onset
hastens recovery as much as PE

26. Recommendations
What Guidelines say ?

27. AAN Guideline
 Treatment with plasma exchange (PE)
or intravenous immunoglobulin (IVIg)
hastens recovery from GBS
 PE and IVIg are equally effective in
patients with advance GBS
symptoms
 PE may carry a greater risk of side
effects and is more difficult to
administer
 Combining the two treatments is not
recommended
 Steroid treatment is not beneficial

28. Endorses the view of AAN related to
IVIg use in GBS
http://www.aanem.org/Education/Patient-Resources/Disorders/Guillain-Barre-

29. NHS – UK
 Two main treatments can be
used to reduce the severity of
Guillain-Barré syndrome They are:
 Intravenous immunoglobulin
 Plasma exchange (plasmapheresis)
 Both treatments are equally
effective.
 Intravenous immunoglobulin is
slightly safer and easier to give
than plasma exchange.
http://www.nhs.uk/Conditions/Guillain-Barre-syndrome/Pages/Treatment.asp

30. Keep on strengthening Nerves……..