International Journal of Neuroscience, 2014; 00(00): 1–6

Copyright © 2014 Informa Healthcare USA, Inc.

ISSN: 0020-7454 print / 1543-5245 online
DOI: 10.3109/00207454.2014.913588

REVIEW

Pathophysiology and diagnosis of Guillain–Barré

syndrome – challenges and needs
Sambit Dash,1 Aparna R. Pai,2 Ullas Kamath,1 and Pragna Rao3
1
Department of Biochemistry, Melaka Manipal Medical College (Manipal Campus); 2 Department of Neurology, Kasturba
Hospital, Manipal, Manipal University; 3 Department of Biochemistry, Kasturba Medical College, India, Manipal
University, Manipal, Karnataka, India

Guillain–Barré syndrome (GBS) is an autoimmune polyneuropathy which presents with acute onset and rapid
progression of flaccid, hyporeflexi quadriparesis. Both sensory and autonomic nerve involvement is seen. GBS
has various subtypes that vary in their pathophysiology. The pathogenesis involves an immune response trig-
gered by a preceding event which may be an infection, immunisation or surgical procedure. Clinical diagnosis
has been largely the primary diagnosing criterion for GBS along with electrodiagnosis, which has several pit-
falls and is supported by ancillary testing of cerebrospinal fluid (CSF) analysis and Nerve Conduction Studies.
Measurement of anti-ganglioside antibodies is also an effective tool in its diagnosis. Further understanding of
pathophysiology and better diagnostic methods are required for better management of GBS.
KEYWORDS: autoimmunity, molecular mimicry, Campylobacter jejuni, anti-ganglioside antibodies, electrodiagnosis

Introduction and could lead to an ascending paralysis. Legs become

French neurologists Georges Guillain, Jean-Alexandre
Barré and André Strohl, in the year 1916, observed
acute areflexic paralysis in two individuals which recov-
ered with time. They found an increased protein con-
centration in the cerebrospinal fluid [1–3]. Called as
Guillain–Barré syndrome (GBS), it was described as a
clinical condition characterised by rapid spread of as-
cending upper or lower limb weakness, defective tendon
reflexes, weak sensory signs and possible autonomic dys-
functions. In the western countries, post-polio eradica-
tion, GBS is a prominent cause of acute flaccid paral-
ysis, bearing resemblance with poliomyelitis and many
other non-specific conditions like AIDS post-surgery,
etc. [4,5]. Inflammation of peripheral nerves, to a larger
extent, causes GBS. Sensory information like pain, tem-
perature is conveyed from the body to the brain and mo-
tor signals like movements, etc., are conveyed from the
brain to the body by these nerves. A tingling sensation
in the legs and arms is felt in GBS. This could be severe
Received 10 February 2014; revised 21 March 2014; accepted 7 April 2014.
Correspondence: Sambit Dash, MSc, Department of Biochemistry, Melaka
Manipal Medical College, Manipal University, Madhavnagar, Manipal,
Karnataka 576104, India. Tel: +91-820-2922519.
E-mail: sambit.dash.cool@gmail.com
weak and the paralysis then sets in the upper limbs and
the face. During this time, there is usually a complete
loss of deep tendon reflexes [6–8].
Infections with a number of bacteria and viruses are
linked to the onset of GBS, of which the most common
is the gram negative diarrhea causing bacteria, Campy-
lobacter jejuni. Autoimmunity which has been a mainstay
in the pathogenesis of GBS involves in it various mecha-
nisms depending on the sub-types of GBS. Greater un-
derstanding of these pathogenesis mechanisms will help
in developing countering strategies. Another area that
needs focus is the diagnostic aspect. While presently
the widely used electrodiagnostic testing is unspecific at
times; an accurate diagnosis and especially early in the
course of the syndrome can help check this often life
threatening syndrome.
Epidemiology
The incidence of GBS is 0.6 to 4 cases per 100,000 pop-
ulation per year across the world [9]. It occurs at all
ages with peaks at adolescence and old age. Below age
40, at an incidence rate of yearly 1.3–1.9 per 100,000
population, the incidence rate remains unvarying. Stud-
ies show a slight increase in incidence during late ado-
lescence and young adulthood. Increased infection risk

1
2 S. Dash et al.
with Cytomegalovirus (CMV) and Campylobacter jejuni
at that age and in the elderly stands as the reason for the
peaks of incidence [10–12]. There is consensus that men
are predisposed to GBS 1·5 times more than women.
Europe and North America see a steady rise in inci-
dence rate in advancing age [13]. Some types of im-
munisations have also been reported to trigger GBS in
susceptible persons. Post “swine flu” vaccination in the
USA in 1976, slight increase in GBS incidence rates oc-
curred [14–16]. Rabies vaccine containing brain mate-
rial is seen to cause GBS at a rate of one in 1000 cases
[17,18]. The lifetime likelihood of any individual acquir-
ing GBS is projected at 1 in 1000 [19].
Preceding events
The relation of preceding illness was not studied by neu-
rologists who defined GBS, namely Guillain, Barré and
Strohl. However, a wide range of clinical observations
aided by epidemiological studies, establish that about
75% of patients report preceding infection as history.
GBS thus could be called a post-infectious illness. The
patients report an antecedent infection which is acute
and which commonly involves respiratory-tract or gas-
trointestinal tract. The infection subsides by the time the
neuropathic symptoms starts. The time taken for GBS
to set in after the preceding infection varies between
one and three weeks and sometimes longer. In several
large studies, the average was 11 days [20]. When cul-
ture studies were done in the serum of patients, about
30% to 50% of cases were found to have antecedent in-
fections [21]. Campylobacter jejuni, a prominent bacteria
causing gastroenteritis worldwide, has been identified as
the organism widely associated with GBS. This link has
been recorded in many studies and in 14 large prospec-
tive studies. C jejuni culture studies have reflected that
the infection causing GBS ranged from 26% to 41%
in a series of isolated GBS cases from countries like
the UK, the Netherlands, the USA, and Japan [22–25].
Case-controlled studies show a significant association of
GBS with not only C jejuni, but also CMV, and probably
Epstein–Barr (EPB) virus [26,27].
Variants
Individual patients with GBS may be classified accord-
ing to whether the myelin sheaths or axons are pri-
marily affected and whether the motor, sensory or au-
tonomic systems are involved. In North America and
Europe, typical patients with GBS usually have AIDP
as the underlying subtype, and only about 5% of the
patients have axonal subtypes of the disease [28]. In
northern China, Japan and Central and South America,
axonal forms are more common [29,30]. In AMSAN,
sensory axons and motor neurons are affected [31]. In
AMAN, the neurological deficit is purely motor [29,30].
Rare cases of acute transient sensory neuropathy may
represent AIDP affecting only sensory nerves or roots
but need to be distinguished from acute sensory neu-
ronopathy. Autonomic involvement is common in GBS,
especially in severe cases with respiratory failure. Some
cases of acute dysautonomia without involvement of so-
matic nerves may be inflammatory and possibly autoim-
mune. In most cases, the nerve supply of the limb, cra-
nial and respiratory muscles is diffusely and symmet-
rically affected; in a minority, the neurological deficit
is much more focused. The most frequent example of
the latter is the Fisher syndrome consisting of ophthal-
moplegia, ataxia and areflexia with variable degrees of
bulbar muscle involvement [31]. Some patients resem-
ble this syndrome at the onset and then develop gen-
eralised weakness, FS/GBS overlap syndrome. There is
also a rare group that develops bulbar involvement, some
of whom also develop upper limb involvement. Most
patients with GBS have a monophasic disease. Recur-
rences occur in less than 3% of patients. Such patients
and those whose illness has a protracted onset phase may
be difficult to distinguish from chronic inflammatory de-
myelinating polyradiculoneuropathy (CIDP). There is
likely to be a spectrum from acute through subacute
to chronic inflammatory demyelinating polyneuropathy,
reflecting the fact that these illnesses share some patho-
genetic mechanisms [32] (Tables 1 and 2).
Pathogenesis
The GBS is considered to be an immune-mediated
polyneuropathy since in the peripheral nerves of these
patients, deposits of complement, immunoglobulins,
and infiltration of macrophages are seen. In the
blood of GBS patients, anti-ganglioside antibodies,
Table 1. Distribution of gangliosides in peripheral nervous
system [59].
Target antigens
of anti-ganglioside
antibodies Localisation in human PNS
GM1 Not determined. In animals axolemma
at node and paranode, dorsal root
ganglia (DRG)
GM1b Not determined
GD1a Motor nerve terminal
GalNac GD 1a Periaxonal membrane of motor nerve at
node and paranode, axolemma of
small fibers in sural nerve
GD1b Large neurons in DRG, paranodal
myelin
GQ1b Paranodal myelin of oculomotor,
trovhlear, and abducens nerves
GT1a Not determined
International Journal of Neuroscience

Table 2. Classification of GBS and related disorders and typical
antiganglioside antibodies by Hughes and Cornblath [3].
Acute inflammatory
demyelinating
polyneuropathy (AIDP) Not known
Acute motor and sensory axonal GM1, GM1b, GD1a
neuropathy (AMSAN)
Acute motor axonal neuropathy GM1, GM1b, GD1a,
(AMAN) GalNac GD 1a
Acute sensory ataxic neuropathy GD1b
Fisher’s syndrome GQ1b, GT1a
inflammatory cytokines and activated T-cells are also
found [33,34]. In 1982, lIyas et al. first reported that
antibodies to gangliosides in serum from a subgroup of
GBS patients were present [35]. Gangliosides are sia-
lylated glycosphingolipids which differ from each other
with respect to the oligosaccharide attached. Ganglio-
sides are found in relatively high concentrations in neu-
ral membranes. Their role possibly is in multiple signal
recognition processes [36,37]. Specific gangliosides are
distributed typically in peripheral nerves; GM1 is con-
centrated in axons, and in myelin of motor nerve fibres,
while GQ1b is majorly found in myelin of oculomotor
nerves [38–40]. The concept of molecular mimicry as
a possible mechanism via which infections may induce
pathogenic immune responses, has been proposed in
many autoimmune diseases [41,42]. The role of molec-
ular mimicry between microbial and autologous anti-
gens in producing a cross-reactive immune response can
be established at different levels: (i) homology in bio-
chemical structure or linear amino acid sequence, (ii)
cross-reactivity of antibodies with both structures, (iii)
cross-reactive antibodies could be induced by process
of immunisation with the microbial antigen and (iv) in-
duction of cross-reactive antibodies which induce dys-
function or tissue damage [42,43].
For a cross-reactive immune response to be initiated
against autologous antigens, by the process of molecu-
lar mimicry, two primary events need to occur. The first
being similarity between the microbial and the autolo-
gous antigen so that immune responses can cross-react.
The second requirement being sufficient dissimilarity of
the microbial antigen from the autologous antigen. This
is required so that the immune systems tolerance to self
is broken and a very specific immune response is gen-
erated. This could occur by affinity maturation of iso-
type switch processes. Lipopolysaccharide (LPS) found
in cell walls of C jejuni strains have been found to in-
duce anti-ganglioside antibodies in some patients. LPS
are cell wall components of most gram-negative bac-
teria. The LPS structure talks for it being responsible
for molecular mimicry. It consists of primarily a lipid

C 2014 Informa Healthcare USA, Inc.
Challenges and Needs 3
A, which anchors the molecule in outer bacterial mem-
brane and a core oligosaccharide and a polymer of re-
peating oligosaccharides. LPS of C jejuni strain contains
in its core oligosaccharide, sialic acids which are charac-
teristics for gangliosides [42,43].
Anti-ganglioside antibodies also play a role in patho-
genesis of GBS. They usually mediate the disease pro-
gression by complement dependent action. In AMAN,
autopsy studies have shown IgG and complement de-
posits on axolemma at the nodes of Ranvier of mo-
tor fibers. Demyelination and lymphatic infiltration is
minimal which is followed by macrophage infiltration.
C jejuni bound to motor nerve terminals destruct neu-
romuscular junction in a complement dependent man-
ner in AMAN. In Miller Fischer syndrome too by clas-
sic complement pathway activation, which forms pores
on membrane attack in presynaptic membrane causing
neuronal and perisynaptic Schwann cell injury. Thus,
complement inhibitors are also being tried for therapeu-
tic uses [59].
Diagnosis
Early diagnosis of GBS is essential as early treatment
decreases the duration of GBS and its severity. It would
also help in reducing GBS patients that would require
mechanical ventilation. Confirmation of diagnosis of
GBS depends on Nerve Conduction Studies (NCS). It
along with electromyography (EMG) are used in diag-
nosis, and different neurophysiological diagnostic crite-
ria have been proposed [39]. NCS serves in differential
diagnosis, to find out the subtype of GBS and to exclude
other mimics. NCS is based on abnormalities in motor
nerves in order to characterise demyelination; sensory
nerve conduction studies help to classify axonal GBS
into AMAN and AMSAN. The ability of NCS to diag-
nose better is enhanced by studying a minimum of three
sensory and four motor nerves. F-waves and H reflexes
may also be studied additionally [40,41].
Clinical diagnostic criteria of GBS have been set but
no such neurophysiological criteria for classification has
been set [28,42–45]. Electrodiagnostic studies help in
the evaluation of patients by helping in diagnosis, classi-
fication and establishing prognosis [28, 46,47]. Abnor-
mal late responses are most common findings in elec-
trodiagnostic studies within 10 days of setting of GBS
[48,49]. Distal temporal dispersion (TD) [50], partial
motor conduction block (CB) [51], abnormal blink re-
flex (BR) [52,50], the presence of multiple A-waves
[53], and a sural-sparing pattern could be used as neu-
rophysiological markers.
Electrodiagnostic findings may not meet neurophys-
iologic criteria in the early stages of GBS [50,54,55].
It though could be said that neurophysiological tests
4 S. Dash et al.

Table 3. Suggested investigations for Guillain–Barré syndrome Table 4. Differential diagnosis of GBS as described by Hughes
as proposed by Hughes and Cornblath[3]. and Cornblath [3].

Studies to establish diagnosis of GBS: Differential diagnosis of GBS:

Following are the list of possible causes overlapping
Electrodiagnostic studies GBS presentation and needs to be ruled out
CSF analysis
Studies in special circumstances: 1. Brainstem encephalitis
Urine porphobilinogen and delta amino levulinic acid 2. Brainstem stroke
HIV testing 3. Peripheral neuropathy
Drug and toxin testing • Guillain–Barré syndromes
Studies to understand the causation: • Post-rabies vaccine neuropathy
Stool culture and serology for C jejuni • Diphtheritic neuropathy
Stool culture for poliovirus • Heavy metals, biological toxins or drug intoxication
M pneumonia • Acute intermittent porphyria
Antibodies to gangliosides • Vasculitic neuropathy
CMV, EPB virus serology • Critical illness neuropathy
• Lymphomatous neuropathy
4. Disorders of muscle
• Hypokalaemia
• Hypophosphataemia
do not remain completely normal in GBS, but it could • Inflammatory myopathy
• Acute rhabdomyolysis
be a possibility. This could be because demyelina- • Trichinosis
tion has occurred but at those sites which are very • Periodic paralyses
proximal, making electrodiagnostic testing difficult [56]. 5. Acute anterior poliomyelitis
Increased cerebrospinal fluid (CSF) protein concentra- • Caused by poliovirus
tion is non-specific and is present in more than two- • Caused by other neurotropic viruses
6. Disorders of neuromuscular transmission
thirds of patients, the protein content tends to remain • Myasthenia gravis
normal in early stages of GBS [57] (Table 3). • Biological or industrial toxins
7. Acute myelopathy
• Space-occupying lesions
• Acute transverse myelitis
Challenges in diagnosis

Differential diagnosis – overlaps

A wide range of disorders have overlapped with clinical
symptoms of GBS and thus have to be carefully ruled
out while diagnosing GBS. The fact that a physician
might encounter rarely cases of GBS in his or her life-
time adds to the complexity of diagnosis (Table 4).
Electrodiagnosis
Uncini et al. [53]describe the pitfall in diagnosis of GBS
with relation to electrodiagnosis; axonal GBS is patho-
physiologically characterised not only by axonal degen-
eration but also by reversible conduction failure at the
axolemma of the node of Ranvier. The lack of differ-
ence among blocks of conduction due to demyelination,
failure of reversible conduction and compound muscle
action trigger potential amplitude reduction depending
on length and may falsely diagnose patients with axonal
GBS as having AIDP. A series of electrophysiological
studies is required for diagnosis of GBS subtypes accu-
rately and for the understanding of pathophysiological
mechanisms of muscle weakness. Trustworthy electro-
diagnostic criteria which take into account the reversible
conduction failure pattern should evolve [47]. Another
study by Kalita et. al. presents variations in sensitivity
of different NCS criteria which ranged from 39.2% to
88.2% in an Indian population [58,59].
Biochemical screening
CSF and serum analysis are carried out as ancillary
testing in GBS. CSF protein rises in more than three-
fourths of patients, with mononuclear cell count which
might be normal or abnormal. However, it remains nor-
mal in the initial days of disease setting in. Analysis
for anti-nuclear antibodies, extractable nuclear antibod-
ies, anti-neutrophil cytoplasmic antibodies, erythrocyte
sedimentation rate, C-reactive protein, electrolyte, urea,
creatinine levels, blood sugar levels and serum delta-
aminolevulinic acid for porphyria are carried out but are
non-specific. Urine porphobilinogen is also sometimes
estimated [8].
The road ahead
The inflammatory pathways involving a host of cy-
tokines and other molecules, both pro- and anti-
inflammatory, and their interplay, suggests the complex-
ity in the disease progress and recovery. The various

International Journal of Neuroscience


subtypes of GBS further complicate understanding of
pathogenesis. A more nuanced and clear approach in
elucidation of various inflammatory pathways will help
in targeting therapeutic applications. Diagnostic chal-
lenges in GBS, an autoimmune disease are many. The
existence of various subtypes and overlap among them
further complicate the diagnosis. The overlap between
acute and chronic inflammatory demyelination adds to
the difficulty in characterisation of GBS. While pri-
marily the physician relies on clinical symptoms, nerve
conduction studies are widely carried out to arrive at a
clinical conclusion. However the non-specificity of elec-
trodiagnosis has been debated. A biochemical test using
CSF is inconvenient owing to the requirement of draw-
ing CSF, furthermore the rise in protein levels is not
significant in many cases. Serum, a convenient sample
to carry out tests has not yet been utilised to charac-
terise specific markers which would help in GBS diag-
nosis. Anti-ganglioside antibodies are being increasingly
identified in serum in GBS patients but the present tech-
nology is expensive and time-consuming. With evolv-
ing technology, better methods of diagnosis, whether by
electrodiagnosis or serum marker analysis, need to be
developed to help in the diagnosis of GBS.
Declaration of Interest
The authors report no conflicts of interest. The authors
alone are responsible for the content and writing of this
paper.
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