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revue neurologique 172 (2016) 770–774
Available online at
ScienceDirect
www.sciencedirect.com
8th meeting of the Société Francophone du Nerf Périphérique
Guillain-Barré syndrome: 100 years on
A. Créange
Service de neurologie, université Paris Est (EA4391), hôpital Henri-Mondor, avenue Maréchal-de-Lattre-de-Tassigny,
94010 Créteil, France
info article
abstract
Article history:
The Guillain-Barré syndrome is associated with acute polyradiculoneuritis for almost one
Received 16 March 2016
century. Its spectrum has considerably been enlarged since its first description. It now
Received in revised form
includes pure motor or sensory syndromes, focal forms, demyelinating and axonal neuro-
4 October 2016
physiological features that characterise excitability dysfunctions, and immunological dif-
Accepted 5 October 2016
ferentiations. We can hope that this improved classification will facilitate development of
Available online 17 November 2016
treatment innovations for a condition that is still a life-threatening condition with a severe
functional prognosis in a significant proportion of cases.
Keywords:
# 2016 Elsevier Masson SAS. All rights reserved.
Guillain-Barré syndrome
Polyradiculoneuritis
Life-threatening
1.
History of Guillain-Barré syndrome
The history of Guillain-Barré syndrome (GBS) runs parallel
with the discovery of the peripheral nervous system. Up to the
second half of the 19th century, injury to the peripheral
nervous system had not yet emerged as a possible cause of
palsy. In 1928, Chomel described an epidemic occurring at the
Marie-Thérèse infirmary in Paris; manifestations included
unpleasant tingling sensations, severe pain, and weakness of
the lower limbs (legs and feet) [1]. For most of the victims, the
severe paralysis generally resolved, though there were a few
cases with a fatal outcome. Sometimes considered as the first
recorded manifestation of GBS, this epidemic was undoubtedly more related to a toxic phenomenon than to inflammation. The characteristic features of the acute paralysis were
published in 1959 by Octave Landry (Jean-Baptiste Octave
Landry de Thézillat – 1826–1865) in an article entitled ‘‘Paralysie
E-mail address: creange@univ-paris12.fr.
http://dx.doi.org/10.1016/j.neurol.2016.10.011
0035-3787/# 2016 Elsevier Masson SAS. All rights reserved.
Ascendante Aiguë’’ (Acute ascending palsy) [2]. He reported five
personal observations and five other cases in the literature. He
described the similar severity of the motor and sensory
manifestations, the progressive decrease in peripheral motricity (loss of deep tendon reflexes) as well as the absence of
sphincter disorders, central nervous system manifestations,
alterations of superior functions, headache, or delusion. This
was the first description of GBS. In his treatise, ‘‘Traité complet
des paralysies’’ (Complete treatise of palsies), Landry mentioned other descriptions made before his. During the second
half of the 19th century, many similar cases were reported,
notably by Pellegrino Lévi in 1865 [3], Westphal in 1876 [4], and
Gowers in 1888 [5].
The final step in the description of GBS was the publication
of an article in 1916 signed by Guillain, Barré and Strohl in
Bulletins et mémoires de la société médicale des hôpitaux de Paris
with the title ‘‘Sur un syndrome de radiculo-névrite avec
hyperalbuminose du liquide céphalorachidien sans réaction cellulaire.
revue neurologique 172 (2016) 770–774
Remarques sur les caractères cliniques et graphiques des réflexes
tendineux’’ (On a radiculo-neuritis syndrome with hyperalbuminosis of the cerebrospinal fluid without cell reaction.
Remarks on the clinical and graphic characteristics of the
tendon reflexes) [6].
Guillain and Barré collaborated in the neurology center of
the 6th army in Amiens. Strohl was a radiologist in the same
military hospital [7]. Their work was published in 1920 in a text
entitled war-related neurology work that briefly described GBS
[8], differentiating Landry’s palsy that developed after antityphoid vaccination and from neurological palsy secondary to
toxic gas exposure.
It was suggested that the fact André Strohl ‘‘strayed’’ from
neurology towards other aspects of medicine such as electrophysiology, radiology, or physical medicine, or his young age –
he was 29 and had just received his degree at the time of the
description of the syndrome – or perhaps his background (he
was from Alsace) would explain why he lost the honors of
history in the description of GBS. In any case, the term GBS was
introduced for the first time in Paris in 1927 by Draganescu and
Claudian who described a radiculoneuritis occurring after
staphylococcal osteomyelitis [9]. The case report was presented at the French Society of Neurology after an introduction by Barré. It was at this time that Strohl’s name was
omitted, both in the description of the syndrome and in the
publication the authors cited.
During the First World War, just after the Battle of the
Somme, the three authors published their palsy cases
observed in two soldiers seen within one month. One can
imagine the conditions under which the three physicians
worked to obtain this history-making observation. In similar
circumstances, Clovis Vincent, head physician of the neurology center of the 9th military region of Tours, developed a
‘‘faradic’’ method called ‘‘torpedoing’’ that consisted in
treating ‘‘paralytics’’ and ‘‘plicaturés’’ (camptocormia) suffering from ‘‘obusite’’ (post-traumatic stress) with 100 mA
electrical shocks that were later qualified as torture [10].
The first soldier was a hussar who developed a stinging
sensation of the lower limbs with muscle weakness without
prior infection. One month later, he exhibited deficiency in the
distal muscles of the legs, abolition of the tendon reflexes, and
moderate sensory deficiency. There was no sphincter disorder. One month later, he had improved and was able to walk
for one hour. He was not returned to the war front, but was
sent to the rear for rest. The second case was much more
acute. An infantry soldier presented with motor deficiency
that made him fall backward when he put on his backpack.
One month later, the deficit improved. Recordings of the
tendon reflexes showed a 2-to-3-fold increased latency and
decreased amplitude. The authors concluded that the conduction velocity of the central part of the reflex had to be severely
hampered, but also considered that the muscle must have
been involved since response to direct muscle percussion was
diminished. But the most important point, the point that drew
attention to these cases, was the increased albumin concentration in the cerebrospinal fluid, despite the lack of any
cellular reaction as seen in ‘‘radiculoneuritis’’. In the first case,
the cerebrospinal fluid assay reported 2.5 g/L (0.85 g/L in the
second case) associated with a normal cell count. The authors
emphasized the importance of this finding that is never
771
Box 1. Synonymes of Guillain-Barré syndrome.
Acute ascending paralysis of Landry.
Acute polyneuritis with fever.
Polyradiculoneuritis with albumin-cytology dissociation.
Acute infectious polyneuritis.
GBS.
Landry Guillain-Barré syndrome.
Landry Guillain-Barré and Strohl syndrome.
Idiopathic polyneuritis.
Acute demyelinating inflammatory polyradiculoneuritis.
observed in pure peripheral nerve injury. They also emphasized the good ‘‘lifelong’’ prognosis (Box 1).
2.
Diagnosis and clinical forms
Over the years, refinements were added to the classification of
GBS. The diagnostic criteria started to include the diverse
presentations observed in different patients. As early as 1938,
then in 1953, Guillain had recognized several forms of GBS and
proposed a clinical classification that took into account four
presentations: the form affecting the lower limbs; the spinal
and brain stem form; the diencephalic form; and the
polyradiculoneuropathy form with perturbed consciousness
[11,12]. Twenty years later, Miller Fisher described the
syndrome that later took on his name and that had a
presentation similar to the diencephalic form described by
Guillain. Meanwhile, Bickerstaff presented a syndrome similar
to polyradiculoneuropathy with perturbed consciousness.
Diagnostic criteria were proposed in 1978 [13]. The different
presentations observed led to the proposal for specific criteria
in 1990 designating pure motor, pure sensory, Miller Fisher,
and other localized forms [14]. A new revision was published
in 2001 taking into account neurophysiological results and
describing several subtypes: sensorimotor; pure motor; Miller
Fisher [15]. The axonal or demyelinating classification can be
associated with these motor or sensorimotor forms.
A collaborative work published in 2011 also took into
account neurophysiological criteria [16]. Even more recently, a
collaborative group examined the possibility of basing diagnosis on clinical elements for the different subtypes of GBS
and Miller Fisher syndrome, the latter syndrome known to
evolve into the other (Table 1) [17].
3.
Neurophysiological classification
This improvement in the clinical classification requires
neurophysiological precisions including descriptions of the
demyelinating and axonal features not apprehended by
diagnostic and prognostic questions. GBS can be axonal
or demyelinating. The subtypes of GBS and Miller Fisher
syndrome also have certain neurophysiological features of the
axonal or demyelinating type, but the axonal or demyelinating
772
revue neurologique 172 (2016) 770–774
Table 1 – Subtypes of Guillain-Barré syndrome and Miller
Fisher syndrome.
Guillain-Barré syndrome
Pure sensory Guillain-Barré syndrome
Pure motor Guillain-Barré syndrome (motor demyelinating
neuropathy)
Bilateral facial palsy with paresthesia
Acute axonal motor neuropathy
Pharyngeal-cervical-brachial weakness
Acute pharyngeal weakness
Paraparetic Guillain-Barré syndrome
Acute motor neuropathy with conduction blocks
Acute axonal sensorimotor neuropathy
Miller-Fisher syndrome
Acute ataxic neuropathy
Acute ophthalmoplegia
Acute ptosis
Acute mydriasis
Bickerstaff encephalitis
Acute ataxia with hypersomnolence
characteristic is not linked with prognosis. Thus, axonal forms
can recover rapidly or progress towards degeneration. Similarly, conduction blocks can resolve rapidly or evolve towards
axonal degeneration. The initial clinical manifestations of GBS
are more the consequence of nerve excitability disorders than
definitive histological alterations of the nerves [18]. Nerve
lesions are related to hormonal, cellular, innate immunity or
adaptive immunity inflammatory mechanisms.
4.
Biology and immunology of GBS
Some of the first messages remain. Thus, many authors still
talk about the albumin-cytology dissociation despite the fact
that for decades the routine technique has been to assay total
protein and not albumin. Today the dissociation is defined by
increased cerebrospinal fluid protein content with a cell
count below 50/mm3 [16]. What is the prognostic value of an
elevated protein level? This question requires a prospective
study.
Immunology findings can also be used to establish
another classification. Certain anti-ganglioside antibodies
are associated with certain GBS phenotypes, e.g. focal forms
of GBS or even Miller Fisher syndrome [17]. More generally,
the anti-ganglioside or anti-nodes of Ranvier antibodies
identified [19] result from a normal immune reaction to an
antigen since the IgM then IgG pattern describes an adaptive
immune response.
5.
Etiological diagnosis
There is a long list of potential differential diagnoses for
GBS. New cases of GBS have been described in recent years.
Thus, infections by various viruses – dengue, hepatitis E,
chikungunya, West Nile, and most recently Zika – can be
implicated.
Clinicians should also remember that acute focal neuropathies corresponding to limited forms of GBS must not be ruled
out too quickly. Certain questions remain open concerning the
differential diagnosis of GBS versus chronic polyradiculoneuritis. These are recurrent forms of GBS exhibiting posttreatment fluctuations, or acute forms of chronic polyradiculoneuritis [20].
6.
Pathophysiology
Two elements must be distinguished. It is important to
recognize that the mechanisms underpinning the inflammation are different from their physiological consequences
affecting the nerve. Certain immunological mechanisms
involve perturbed innate immunity plus elements of
adaptive immunity. For innate immunity, dendritic cells,
complement fractions, mast cells, monocytes/macrophages,
and certain toll-like receptors with a favoring effect play an
important role. Early activation of adaptive immunity is
involved, as shown by IL17 and IL22 secretion,
CD4(+)CD25(+) regulator T-cell anomalies, and, of course,
the presence of anti-ganglioside and anti-nodes of Ranvier
protein IgG. This immune reaction results from the
encounter with an antigen that has passed the first line
of defense, i.e. the pharyngeal or digestive mucosa. Several
arguments are in favor of a cross-reactivity phenomenon,
especially in the cases of Campylobacter jejuni diarrhea with
certain glycated (anti-GM1) or protein nerve antigens [21].
Other humoral factors of innate immunity and adaptive
immunity participate in the nerve lesions, e.g. pro-inflammatory cytokines (TNFa).
For the nerve, the consequences are multifocal demyelinating lesions developing via cellular (macrophage) or
hormonal (cytokines, proteinases) mechanisms. The lesions
may be focal, affecting the nodes of Ranvier and the
neuromuscular junction (nerve endings) leading to excitability
disorders related to anti-ganglioside antibodies, antibodies
with no specifically recognized antigen, or anti-protein antibodies (nodo-paranodopathy) [19].
7.
Prognosis
The idea that the prognosis is good was important for the early
authors and remains so today despite reports of mortality
rates above 4% in expert centers, disability rates reaching
nearly 15%, and cases of prolonged disability with diminished
strength, pain, fatigue, and need for occupational readjustments in 40% of cases [22].
Several elements affecting prognosis have been identified
including older age, diarrhea as a triggering factor, important
motor deficit and disability (MRC < 30), hospital admission,
short interval between motor symptom onset and hospital
admission, mechanical ventilation, and absence of motor
action potential on neurophysiological recordings [22]. High
serum glucose levels are associated with more severe
disease.
revue neurologique 172 (2016) 770–774
Simple clinical scores (e.g. EGRIS) can be determined at
admission to assess the short-term risk of intubation. EGRIS
combines items for older age, rapid disease progression,
disease severity (GBS score), presence of triggering diarrhea,
positive Campylobacter jejuni or cytomegalovirus serology, or
the absence of prior respiratory morbidity, as well as inability
to raise the head off the bed, elevated transaminase level, and
vital capacity < 60% [23,24]. For the mid- and long-term
prognosis, the EGOS (determined two weeks after admission)
and the mEGOS (determined at one week) provide an
assessment of the capacity to walk at 6 months or 4 weeks,
respectively [25,26].
8.
Treatment
No revolution has taken place in the treatment of GBS in the
recent years. Intravenous immunoglobulins or plasma
exchange remain the mainstay treatments. Two studies
conducted in Europe and Japan are under way to test
eculizumab, a complement anti-activator. There remains
the question of the usefulness of a second immunoglobulin
infusion for GBS. It is also raised in the case of a suspected
chronic demyelinating neuropathy with an acute onset.
Despite the absence of evidence from a comparative trial, a
second infusion could be proposed if the baseline and postinfusion IgG concentrations vary little [27] or if in the presence
of post-treatment clinicalseverity fluctuations [28].
It was the advent of the first laboratory tests that led to the
definition of GBS. Making its way through the 20th century,
GBS took a lot of discussion, and a few battles among
international experts – and among the different schools of
thought in France – to finally achieve full recognition. In an era
when the number of diseases known by their eponym is
declining, it must be remembered that GBS probably would not
exist without the strong will of its first two authors. Today GBS
englobes a large spectrum of syndromes illustrated by a
century of clinical, neurophysiological and immunological
refinements. For the upcoming years, we can hope that the
significant progress recently made in our understanding of the
pathophysiological mechanisms underpinning GBS will
enable more targeted and more effective treatments.
Disclosure of interest
The author received departmental research grants from
Biogen-Idec, CSL-Behring, GE Neuro, Novartis, Octapharma
and expert testimony from Novartis, CSL Behring, Genzyme.
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