Autoimmunity Reviews 16 (2017) 96101

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Autoimmunity Reviews

journal homepage: www.elsevier.com/locate/autrev

Review

GuillainBarr syndrome
Susanna Esposito , Maria Roberta Longo
Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Universit degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy

a r t i c l e i n f o a b s t r a c t

Article history: The term GuillainBarr syndrome (GBS), the most frequent cause of acute paralytic neuropathy, covers a num-
Received 8 August 2016 ber of recognisably distinct variants. The exact cause of GBS is unknown, but 5070% of cases appear 12 weeks
Accepted 15 September 2016 after a respiratory or gastrointestinal infection, or another immune stimulus that induces an aberrant autoim-
Available online 23 September 2016
mune response targeting peripheral nerves and their spinal roots. The interplay between the microbial and
host factors that dictate whether and how the immune response shifts towards autoreactivity is still unclear,
Keywords:
Acute inammatory demyelinating
and nothing is known about the genetic and environmental factors that affect an individual's susceptibility to
polyneuropathy the disease. All patients with GBS need meticulous monitoring, and can benet from supportive care and the
Acute motor axonal neuropathy early start of specic treatment. This review summarises the clinical features and diagnostic criteria of GBS and
Acute paralytic neuropathy proposes an algorithm for its management. An analysis of the literature showed that, about one century after it
GuillainBarr syndrome was rst described, new information concerning its etiopathogenesis has allowed the development of new treat-
ment strategies that should be started immediately after diagnosis; however, the available therapies are not suf-
cient in many patients, especially in the presence of the acute inammatory demyelinating polyneuropathy.
New post-infectious forms, such as those caused by Zika virus and enterovirus D68, need to be carefully analysed
and, in order to improve patient outcomes, research should continue to aim at identifying new biomarkers of dis-
ease severity and better means of avoiding axonal injury.
2016 Elsevier B.V. All rights reserved.

Contents

1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96
2. Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97
3. Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98
4. Clinical features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98
5. Diagnostic algorithm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98
6. Therapeutic approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99
7. Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99
Take-home messages . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99
Acknowledgement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100

1. Introduction weakness, unstable ambulation, and hypo- or areexia [2,3]. The weak-
ness is usually predominantly distal, at least at the time of onset, and
GuillainBarr syndrome (GBS), the most frequent cause of acute many patients feel neuropathic pain. GBS usually presents as ascending
paralytic neuropathy [1], is an inammatory polyneuropathy that is paralysis, with weakness in the legs spreading to the upper limbs and
characterised by an acute onset, rapid progression, symmetric muscle the face, and the complete loss of deep tendon reexes [4,5]; however,
there are a number of recognisably distinct variants [6].
The exact cause of GBS is unknown, but 5070% of cases appear 1
Corresponding author at: Pediatric Highly Intensive Care Unit, Department of
Pathophysiology and Transplantation, Universit degli Studi di Milano, Fondazione
2 weeks after a respiratory or gastrointestinal infection, or another im-
IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122 Milano, Italy. mune stimulus that induces an aberrant autoimmune response targeting
E-mail address: susanna.esposito@unimi.it (S. Esposito). peripheral nerves and their spinal roots [2,3,7]. The interplay between the

http://dx.doi.org/10.1016/j.autrev.2016.09.022
1568-9972/ 2016 Elsevier B.V. All rights reserved.
 S. Esposito, M.R. Longo / Autoimmunity Reviews 16 (2017) 96101
microbial and host factors that dictate whether and how the immune re-
sponse shifts towards autoreactivity is still unclear, and nothing is known
about the genetic and environmental factors that affect an individual's
susceptibility to the disease [6]. When patients present with rapidly pro-
gressive paralysis, GBS needs to be diagnosed as soon as possible. All pa-
tients with GBS need meticulous monitoring, and can benet from
supportive care and the early start of specic treatment [8].
This review summarises the clinical features and diagnostic criteria
of GBS and proposes an algorithm for its management. The MEDLINE
and PubMed databases were searched for all of the studies published
over the last 15 years using the key words GuillainBarr syndrome.
Only the articles published in English were included in the analysis.
2. Epidemiology
The widespread eradication of poliomyelitis has led to GBS becom-
ing the most frequent cause of acute and sub-acute accid paralysis,
with a reported annual incidence of 0.52 cases per 100,000 people [9,
10]. The annual incidence rate increases with age, and GBS is rare in chil-
dren aged b 2 years [11]. Men are about 1.5 times more likely to be af-
fected than women [1214].
Table 1 summarises the main etiological factors associated with GBS.
The differences in the incidence of GBS in different populations may re-
ect variations in genetic susceptibility or in the exposure to causative
pathogens. Although many different infections have been identied in
patients with GBS, casecontrol studies have revealed associations
with only a few pathogens. Campylobacter jejuni is the most widely re-
ported infection: it has been found in 2550% of adult patients, and is
more frequent in Asian countries [15,16]. Other infections associated
with GBS are those due to cytomegalovirus, EpsteinBarr virus, measles,
inuenza A virus and Mycoplasma pneumoniae [1721], as well as en-
terovirus D68 [22] and Zika virus [2326].
Recently, Williams et al. reported a cluster of atypical GBS in 10
adults temporally related to a cluster of four children with acute accid
paralysis, over a 3-month period (from October 2015 to January 2016)
in South Wales, United Kingdom [22]. All adult cases were male, aged
between 24 and 77 years. Seven had prominent facial diplegia at
onset. Available electrophysiological studies showed axonal involve-
ment in ve adults. Seven reported various forms of respiratory disease
before onset of neurological symptoms.
The rst report of GBS associated with Zika virus infection was re-
ported in French Polynesia between October 2013 and April 2014 [23].
A total of 41 (98%) patients with GBS had anti-Zika virus IgM or IgG,
and all (100%) had neutralising antibodies against Zika virus compared
with 54 (56%) of 98 controls (p b 0.0001). 39 (93%) patients with GBS
had Zika virus IgM and 37 (88%) had experienced a transient illness in
a median of 6 days before the onset of neurological symptoms, suggest-
ing recent Zika virus infection. Patients with GBS had electrophysiolog-
ical ndings compatible with acute motor axonal neuropathy type, and
had rapid evolution of disease. 12 (29%) patients required respiratory
Table 1
Main etiological factors associated with GuillainBarr syndrome.
Infections Bacteria
Campylobacter jejuni
Mycoplasma pneumoniae
Viruses
Cytomegalovirus
EpsteinBarr virus
Inuenza A virus
Enterovirus D68
Zika virus
Vaccines Rabies vaccine
Inuenza A/H1N1 vaccine (association
conrmed only during the 1976 campaign)
97
assistance. No patients died. Anti-glycolipid antibody activity was
found in 13 (31%) patients.
In a recent study, Lucchese and Kanduc analysed Zika virus
polyprotein for peptide sharing with human proteins that, when al-
tered, associate with microcephaly and brain calcications [25]. Results
highlighted a vast viral versus human peptide commonality that, in par-
ticular, involves centriolar and centrosomal components canonically
cataloged as microcephaly proteins, i.e., C2CD3, CASC5, CP131, GCP4,
KIF2A, STIL, and TBG. Likewise, a search for Zika virus peptide occur-
rences in human proteins linked to GBS also show a high, unexpected
level of peptide sharing. Of note, further analyses using the Immune Epi-
tope Database resource show that many of the shared peptides are
endowed with immunological potential. These data indicate that im-
mune reactions following Zika virus infection might be a considerable
source of cross-reactions with brain-specic proteins and might con-
tribute to the Zika virus-associated neuropathologic sequelae [25].
GBS has been observed in subjects who had recently been vaccinated
against rabies [27] and inuenza A virus [19,28,29]. During the 1976
vaccination campaign against A/H1N1 inuenza, one out of 100,000
people who had been vaccinated developed GBS [27]; however, this
was not the case in subsequent years [6]. To assess the effect of seasonal
inuenza vaccination on the absolute risk of acquiring GBS, Hawken
et al. used simulation models and published estimates of age- and sex-
specic risks for GBS, inuenza incidence, and vaccine effectiveness
[19]. For a hypothetical 45-year-old woman and 75-year-old man, ex-
cess GBS risk for inuenza vaccination versus no vaccination was
0.36/1 million vaccinations (95% credible interval 1.22% to 0.28)
and 0.42/1 million vaccinations (95% credible interval, 3.68 to
2.44), respectively. These numbers represent a small absolute reduction
in GBS risk with vaccination. Under typical conditions (i.e. inuenza in-
cidence rates N5% and vaccine effectiveness N 60%), vaccination reduced
GBS risk. These ndings should strengthen condence in the safety of
inuenza vaccine and allow health professionals to better put GBS risk
in context when discussing inuenza vaccination with patients [19].
Haber et al. indicated that, with rare exceptions, associations between
vaccines and GBS have been only temporal [30]. There is little evidence
to support a causal association with most vaccines. The evidence for a
causal association is strongest for the swine inuenza vaccine that was
used in 197677. Studies of inuenza vaccines used in subsequent
years, however, have found small or no increased risk of GBS. Older for-
mulations of rabies vaccine cultured in mammalian brain tissues have
been found to have an increased risk of GBS, but newer formulations
of rabies vaccine, derived from chick embryo cells, do not appear to be
associated with GBS at a greater than expected rate. In an earlier review,
the Institute of Medicine concluded that the evidence favoured a causal
association between oral polio vaccine and tetanus toxoid-containing
vaccines and GBS. However, recent evidence from large epidemiological
studies and mass immunization campaigns in different countries found
no correlation between oral polio vaccine or tetanus toxoid-containing
vaccines and GBS. Spontaneous reports to the US Vaccine Adverse
Events Reporting System shortly after the introduction of quadrivalent
conjugated meningococcal vaccine raised concerns of a possible associ-
ation with GBS. Comparisons with expected rates of GBS, however, were
inconclusive for an increased risk, and lack of controlled epidemiologi-
cal studies makes it difcult to draw conclusions about a causal associa-
tion. For other vaccines, available data are based on isolated case reports
or very small clusters temporally related to immunizations, and no con-
clusion about causality can be drawn [30].
There are certain circumstances in which immunizing individuals,
particularly those with a prior history of GBS, may require caution. How-
ever, the benet of vaccines in preventing disease and decreasing mor-
bidity and mortality, particularly for inuenza, needs to be weighed
against the potential risk of GBS. Generally, the vaccination is not contra-
indicated in patients who have previously had the disease unless it was
vaccination related or occurred during the previous three months; how-
ever, the risks and benets should be discussed on a case by case basis [6].
98 S. Esposito, M.R. Longo / Autoimmunity Reviews 16 (2017) 96101
3. Pathogenesis
GBS was long considered a homogeneous disorder whose severity
was related to the extent of axonal injury arising from demyelinisation;
however, it is now known that there are various phenotypes, including
acute inammatory demyelinating polyneuropathy (in which the
immune-related injury affects the myelin sheath and related Schwann
cells) and acute motor axonal neuropathy, in which the membranes of
nerve axons are the primary target [6].
GBS occurs in healthy people, and is not typically associated with an
autoimmune or other systemic disorder. It is a mainly humoral-
mediated rather than T-cell mediated disorder [31] and, in this context,
acute motor axonal neuropathy appears as an antibody-mediated attack
driven by molecular mimicry between microbial (i.e. glycans) and
axolemmal surface molecules (i.e. GM1 and GD1a gangliosides) [32,
33]. On the contrary, the immunological mechanisms involved in
acute inammatory demyelinating polyneuropathy is less clear because
of the wide range of immune stimulants that can cause it and the ab-
sence of specic antibody biomarkers [6,33].
Although the distinction between acute motor axonal neuropathy
and acute inammatory demyelinating polyneuropathy seems to be
conceptually clear, the margins between the two conditions are not so
well dened. Electrophysiological ndings are often ambiguous as
they indicate acute inammatory demyelinating polyneuropathy in
the early phases and acute motor axonal neuropathy later, or both con-
ditions simultaneously [34].
An interesting mechanism that could have a role in GBS is represent-
ed by the T cell epitope redundancy described by Moise et al. [35]. T cells
are extensively trained on self in the thymus and then move to the pe-
riphery, where they seek out and destroy infections and regulate im-
mune response to self-antigens. T cell receptors (TCRs) on T cells'
surface recognise T cell epitopes, short linear strings of amino acids
presented by antigen-presenting cells. Some of these epitopes activate
T effectors, while others activate regulatory T cells. It was recently dis-
covered that T cell epitopes that are highly conserved on their TCR
face with human genome sequences are often associated with T cells
that regulate immune response. These TCR-cross-conserved or redun-
dant epitopes are more common in proteins found in pathogens that
have co-evolved with humans than in other non-commensal pathogens.
Epitope redundancy might be the link between pathogens and autoim-
mune disease [35].
4. Clinical features
In 1916, the French neurologists Guillain, Barr, and Strohl described
two soldiers who developed acute paralysis with areexia who sponta-
neously recovered. They noted increased protein concentration with a
normal cell count in the cerebrospinal uid (CSF) [36]. The combination
of these clinical and laboratory features became known as the GBS. In
recent decades it has become clear though that GBS contains a spectrum
of acute idiopathic, usually monophasic, peripheral neuropathies [37].
The symptoms at the time of onset of classic GBS are pain, paresthe-
sia, numbness, and rapidly progressive bilateral limb weakness [6,38,
39]. Patients generally manifest rubbery legs or legs that tend to buck-
le, with or without numbness or tingling. The weakness usually starts in
the distal lower extremities and progresses upwards over a period of
hours or days, until the arms and facial muscles also become affected,
leading to bulbar weakness and respiratory difculties [40]. The muscle
weakness sometimes develops in the arms rst (descending type), or si-
multaneously in the arms and legs. In some forms of the disease, such as
Miller Fisher syndrome, the weakness may only affect the cranial
nerves, leading to facial, oculomotor, or bulbar weakness [41]. The
weakness reaches its peak 24 weeks after symptom onset. A small
number of patients present with paraparesis, which may remain during
the course of the disease [42].
In addition to weakness, patients may present sensory signs, ataxia,
and autonomic dysfunction [6]. Muscle pain or radicular pain, which
precedes the weakness in about 30% of patients, is another frequent ini-
tial sign [6]. Reexes may initially be normal in pure motor and axonal
forms, or sometimes even hyper-reexic [43].
The disease can progress for up to six weeks after onset [6], during
which 2030% of patients develop complications, including respiratory
failure requiring mechanical ventilation [44]. Further complications
are aspiration pneumonia, sepsis, cardiac arrhythmia (i.e., tachycardia
or bradyarrhythmia with asystole), arterial hypertension or hypoten-
sion, abnormal sweating, and gastrointestinal dysmotility [8]. Urinary
retention and constipation are unusual at the onset of the disease but
commonly develop at the nadir of the disease [8]. Signs of autonomic
dysfunction are more frequent in patients with severe weakness and re-
spiratory failure [8].
Most cases in North America and Europe are caused by acute inam-
matory demyelinating polyradiculoneuropathy (AIDP) [45]. Axonal
forms which are more frequent in Asia and Japan consist of a purely
motor form which is called acute motor axonal neuropathy (AMAN)
[46] and a form when sensory bers are also affected which is then
called acute motor and sensory axonal neuropathy (AMSAN) [47].
Other rare variants of the GBS spectrum are Miller Fisher syndrome
(MFS) [48] which typically exhibits a triad of ophthalmoplegia, areexia
and ataxia, Bickerstaff brainstem encephalitis [50] which starts with
cranial nerve dysfunction and can progress to brainstem encephalitis,
pharyngocervico-brachial pattern, and pure sensory form. The MFS var-
iant of GBS accounts for 5% of cases in Europe, and higher percentages in
Japan and Taiwan [49].
GBS can be difcult to diagnose in young children because they
present their complaints atypically, and a neurological examination is
more challenging [51]. The differential diagnosis is very wide and exclu-
sion of alternative causes such as brainstem or spinal cord lesion, neuro-
muscular junction defect or muscular abnormalities is necessary [37].
Once the diagnosis of an acute peripheral neuropathy is established,
GBS is the most common, but not the only cause and the clinician should
consider alternative causes such as toxic neuropathy, porphyria, vascu-
litis, tick paralysis and metabolic disturbances.
Overall, GBS is a life-threatening disease with a mortality rate of 3
7% [52,53]. Patients mainly die of ventilatory insufciency, pulmonary
complications or autonomic dysfunction. Those who survive are quite
frequently affected by residual complaints and decits that have a sub-
stantial effect on their everyday activities and quality of life [54]. Im-
provements mainly occur during the year after GBS onset, although
patients may recover further even after three years or more. Poor out-
comes are associated with an older age (40 years), diarrhea or C. jejuni
infection in the four weeks preceding the disease, and a high degree of
disability when the weakness is maximal [6].
5. Diagnostic algorithm
Table 2 summarises the criteria for a diagnosis of GBS, which is
mainly based on the results of a clinical examination, but additional in-
vestigations may be required for conrmation [55].
Most patients with suspected GBS undergo a lumbar puncture. Their
cerebrospinal uid (CSF) typically shows cyto-albuminological dissoci-
ation (i.e. a normal cell count with increased protein levels) [56]. CSF
protein concentration is often normal in the rst week after onset, but
increases in more than 90% of patients by the end of the second week
[6]. About 15% of patients have a slightly increased CSF cell count (10
30 cells/L) [8].
Nerve conduction studies (NCS) are usually normal early in the
course of the disease, but that does not exclude a diagnosis of GBS [57,
58] as nerve conduction abnormalities are most pronounced two
weeks after the weakness starts. NCS ndings can sometimes distin-
guish the axonal and demyelinating subtypes of GBS: patients with ax-
onal neuropathy show decreased motor and/or sensory amplitudes,
 S. Esposito, M.R. Longo / Autoimmunity Reviews 16 (2017) 96101
Table 2
Diagnostic criteria of GuillainBarr syndrome.
Clinical features Progressive weakness over a period of up to 6 weeks in legs
and arms (sometimes only in arms)
Hypo- or areexia (sometimes normal or even hyper-reexia)
Relative symmetry
Mild sensory symptoms or signs
Pain
Autonomic dysfunction
Complications such as respiratory failure requiring mechanical
ventilation, aspiration pneumonia, sepsis, cardiac arrhythmia,
hyper- or hypotension and urinary retention
Lumbar puncture Cytoalbuminological dissociation (i.e. normal cell count with
increased protein levels) in cerebrospinal uid
Nerve conduction Evident after 2 weeks, showing decreased motor and/or
studies sensory amplitudes
In the case of demyelinating polyneuropathy, prolonged
distal motor latency, reduced nerve conduction velocity,
prolonged F-wave latency, increased temporal dispersion,
and conduction blocks
Magnetic resonance Post-gadolinium enhancement of the peripheral nerve roots
imaging and cauda equine
Serum Anti-ganglioside antibodies (in about 50% of patients)
whereas those with demyelinating polyneuropathy show signs such as
prolonged distal motor latency, reduced nerve conduction velocity,
prolonged F-wave latency, increased temporal dispersion, and conduc-
tion blocks. They may also relate to prognosis insofar as patients with
demyelination more often need mechanical ventilation, and low com-
pound muscle potentials are the most consistent ndings predicting a
poor outcome [6,59].
Neuroimaging has become a valuable adjunct to NCS in the case of
suspected GBS. Although it is not specic, post-gadolinium enhance-
ment of peripheral nerve roots and the cauda equine can be seen on spi-
nal magnetic resonance imaging (MRI) scans of as many as 95% of
patients [60,61]. Furthermore, MRI can exclude structural myelopathy
or a para-sagittal cerebral lesion.
Finally, anti-ganglioside antibodies can be identied in about 50% of
patients, and can be very useful in conrming a diagnosis, particularly in
the case of patients with atypical presentations [62].
6. Therapeutic approach
The treatment of GBS requires a multidisciplinary approach consisting
of general medical care and immunological treatment (Table 3). The
monitoring of respiratory, cardiac and hemodynamic function is needed
to prevent or manage complications [6], and care should also be taken
to ensure prophylaxis for deep vein thrombosis, the management of pos-
sible bladder and bowel dysfunction, the early start of physiotherapy and
rehabilitation, and psychosocial support [6,63,64]. In addition, pain man-
agement with opioids or non-steroidal anti-inammatory drugs is ex-
tremely important [68].
Table 3
Therapeutic approaches to GuillainBarr syndrome.
General medical care Monitoring of respiratory, cardiac and hemodynamic
function
Prophylaxis for deep vein thrombosis
Management of possible bladder and bowel dysfunction
Early initiation of physiotherapy and rehabilitation
Psychosocial support
Pain management using opioids or non-steroidal
anti-inammatory drugs
Immunological treatment Intravenous immunoglobulin therapy
with documented efcacy Plasma exchange
New treatments under Interferon-beta
evaluation Cyclophosphamide
Rituximab
Eculizumab
99
Although there is no specic drug for GBS, a number of drugs have
been used to target the components of the immune response.
Immunomodulating treatments, mainly in the form of intravenous im-
munoglobulin (IVIg) therapy and plasma exchange (PE), have proved to
be efcacious in hastening recovery and improving outcome [65]. Both
IVIg and PE should be started as soon as possible, before irreversible
nerve damage has taken place. It is not known whether the
total IVIg dose of 2 g/kg on two days is more benecial than a dose of
0.4 g/kg/day for ve days. Five PE sessions over two weeks are usually rec-
ommended but, in many centres, IVIg therapy is preferred because of its
widespread availability and good tolerability, although it is more expen-
sive than PE. Combined PE and IVIg is no better than PE or IVIg alone,
and there is no evidence in favour of a second course of IVIg [6].
Oral and intravenous steroids, alone or in combination with IVIg or
PE, have not been found to be efcacious in patients with GBS [66].
Some new immunological treatment strategies are currently being
investigated. These include interferon (IFN)-beta treatment, which de-
creases the number of relapses of multiple sclerosis, but its effects in
GBS patients remain controversial [6]. Two case reports suggest that
IFN-beta combined with IVIg is therapeutically benecial [67]. Cyclo-
phosphamide (CY) is an immunomodulator that seems to improve the
clinical and histological features of GBS in an animal model, but
prolonged CY treatment has been associated with infections and neo-
plastic diseases [66]. Another approach is based on humanised mono-
clonal antibodies: rituximab is an antibody against CD20 that can
induce targeted B cell depletion, and eculizumab is an antibody with
high afnity for complement factor C5 [6,68]. However, no clinical
trial has yet documented the efcacy and safety of monoclonal antibod-
ies in GBS patients.
7. Conclusions
About one century after it was rst described, new information
concerning the etiopathogenesis of GBS has allowed the development of
new treatment strategies that should be started immediately after diag-
nosis; however, the available therapies are not sufcient in many patients,
especially in the presence of the acute inammatory demyelinating
polyneuropathy. In addition, new post-infectious forms, such as those
caused by Zika virus and enterovirus D68, need to be carefully analysed
and, in order to improve patient outcomes, research should continue to
aim at identifying new biomarkers of disease severity and better means
of avoiding axonal injury.
Take-home messages
Guillan-Barr syndrome (GBS) is the most frequent cause of acute
paralytic neuropathy.
When patients present with rapidly progressive paralysis, GBS needs
to be diagnosed as soon as possible.
All patients with GBS need meticulous monitoring, and can benet
from supportive care and the early start of specic treatment.
Available therapies are not sufcient in many patients, especially
in the presence of the acute inammatory demyelinating
polyneuropathy.
In order to improve patient outcomes, research should continue to
aim at identifying new biomarkers of disease severity and better
means of avoiding axonal injury.
Acknowledgement
This review was supported by a grant from the Italian Ministry of
Health (Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico
Ricerca Corrente 2016 850/01). The authors declare that they have no
potential conict of interest.
100 S. Esposito, M.R. Longo / Autoimmunity Reviews 16 (2017) 96101
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