polish annals of medicine 21 (2014) 158–161

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Review Article

Guillain–Barré syndrome – Literature overview

Daniel Kopytko a, Piotr M. Kowalski b,*

a
Outpatient Healthcare Clinic Medyk in Młynary, Elbląg, Poland
b
Medical Academy, Lithuanian University of Health Sciences, Kaunas, Lithuania

article info abstract

Article history: Introduction: Guillain–Barré syndrome (GBS) is one of the most prevalently acquired poly-
Received 1 July 2014 neuropathies. In the past, once regarded as separate disease, now it is described as a group of
Accepted 17 July 2014 few acute neuropathy subtypes of autoimmune origin. Although this disease may occur at
Available online 15 August 2014 any stage of life, equally affecting both women and men, the risk increases with age and is
relatively low in children.
Keywords: Aim: Aim of this work is to present pathogenesis, clinical picture, as well as current
Guillain–Barré syndrome (GBS) diagnostic methods and treatment of GBS.
Inflammatory polyneuropathy Discussion: Although GBS is usually preceded by a mild virus infection, sometimes it is
Paresis associated with a bacterial infection affecting either respiratory or digestive system. Initial
Areflexia symptom of classic form of GBS is usually a symmetrical paresis of proximal part of lower
Demyelination limbs, which gradually expands affecting upper limbs and trunk muscles. In case of
diaphragmatic and intercostal nerves involvement, muscle weakness eventually leads to
respiratory failure. As paralysis continues, deep reflexes tend to weaken and disappear.
Diagnosis of GBS is carried out on the basis of clinical picture, cerebrospinal fluid analysis
and electrophysiological study. The range and type of treatment mainly depend on severity
of clinical signs and a phase of the disease.
Conclusions: Diagnosis and treatment of GBS are crucial issues in clinical practice, because
approximately 25% of patients can develop respiratory failure, significant disability followed
by GBS present in 20%, and chronic fatigue in 60%–70% of patients. Despite symptomatic
treatment and immunotherapy, mortality associated with GBS still ranges from 4% to 15%.
# 2014 Warmińsko-Mazurska Izba Lekarska w Olsztynie. Published by Elsevier Urban &
Partner Sp. z o.o. All rights reserved.

of the disease.1 GBS is one of the most commonly acquired

1. Introduction
polyneuropathies. In the past, once regarded as a separate
disease, now it is rather described as a group of few acute
Guillain–Barré syndrome (GBS), or otherwise Landry–Guillain– neuropathy subtypes of autoimmune origin. Incidence of GBS
Barré–Strohl syndrome, was described in 1916. Haymaker and in Poland is about 1.5–4 persons per 100 000 population, which
Kernohan elaborated on clinical and histopathological picture accounts for about 800 new cases per year. Frequency of GBS in

* Correspondence to: Janowicza 32/13, 10-690 Olsztyn, Poland. Tel.: +48 508 110 789.
E-mail address: piotr.kowalski@hotmail.com (P.M. Kowalski).

http://dx.doi.org/10.1016/j.poamed.2014.07.010
1230-8013/# 2014 Warmińsko-Mazurska Izba Lekarska w Olsztynie. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.
 polish annals of medicine 21 (2014) 158–161 159

children is approx. 0.5–1.5 over 100 000 population – charac- 3.1. Signs and symptoms
terized by a milder course of the disease, however CNS
symptoms like dizziness, headaches, optic disk swelling, or Neurodeficiency symptoms usually appear within first 2–28
positive meningeal signs are more prevalent.2 This disease can days and the course of a disease occurs in a single phase
occur at any stage of life, equally affecting both women and fashion in 90% of patients; the remaining group of patients
men, nevertheless some reports indicate that lately it is men develop a chronic or recurrent condition. In spite of general
who suffer more often. The risk of developing GBS increases good prognosis in 20% of patients, development of respiratory
with age and while frequency of poliomyelitis has decreased, failure is highly probable. Death occurs in 3%–5% of patients
GBS became the most frequent acute disease that leads to (some references state 4%–15% mortality rate) usually due to
paresis in Western countries. Despite intensive treatment, GBS cardiovascular complications. Relapses are common and
mortality ranges form 4% to 15%.3,4 frequently may follow infections or vaccinations, even many
years (4–36) after the first episode. Between relapses of the
disease neurodeficiency sustained or patients were complete-
2. Aim
ly free of any symptoms.1,13–15
Pain occurring few days after infection and confined to
Aim of this paper is to present pathogenesis, clinical picture, as interscapular and lumbar region could be very informative
well as current diagnostic methods and treatment of GBS. about the onset of the disease as it is associated with nerve
roots swelling and meningeal irritation. In this period it is
possible to observe in patients neck stiffness and positive
3. Discussion Kernig's sign. Some patients complain about painful pares-
thesia and hypoesthesia, sometimes preceding occurrence of
GBS has been divided into few types accommodating motor signs.16 Paresis usually affects lower limbs first, often in
differences in the pattern of paresis, function of affected proximal part; it gradually expands affecting upper limbs and
fibers, as well as pathologic process. Classic form of GBS is an trunk muscles. Intercostal and diaphragmatic nerves involve-
acute inflammatory demyelinating polyradiculoneuropathy – ment leads to respiratory insufficiency. As paresis progresses,
around 90% of all cases in Europe and USA. Acute motor axonal deep reflexes diminish. After the period characterized by
neuropathy without features of demyelination and with increase of symptoms severity (till 3 weeks in 80% of patients)
damage to motor nerves in Europe accounts for approx. 5% a plateau phase comes around (10–14 days) followed by
of all cases; meanwhile in China this course of the disease is remission phase that lasts 6–14 months in case of severe
characteristic for 70% of GBS.5 Acute motor and sensory axonal paresis. Approximately 30%–50% of patients develop cranial
neuropathy with motor and sensory nerve involvement is nerves involvement (facial, glossopharyngeus, vestibuloco-
associated with more severe course of the disease. Miller- chlear, oculomotor, trigeminus). Disturbance of propriocep-
Fisher syndrome accompanied by ophthalmoplegia, ataxia tion (alignment, vibration) is more frequent rather than
and areflexia is a rather rare form of disease. disturbance of superficial sensation (subjective and objective).
During the course of GBS, damage to nerves occurs through Patients experience signs of radiculopathy and myalgia. In
autoimmunologic mechanisms. Simplifying, destruction is about 30% of patients autonomic symptoms are present and
based on demyelination in classical form of GBS and on should they apply to cardiovascular system, a direct threat to
damage of axons in initial axonal form. Ultimately it has been life is created especially for elderly patients.17–19
proved that activated lymphocytes T and antibodies, especial-
ly those against gangliosides contribute to the pathogenesis of 3.2. Diagnosis
the disease.6
In 75% of patients, GBS morbidity is usually preluded by Diagnosis of GBS mostly relies on clinical picture (progressive
bacterial or virus infection of either respiratory or digestive paresis of lower and upper limbs, sensation loss, cranial
tract, few weeks prior to occurrence of first neurological nerves involvement, especially facial, autonomic dysfunction),
signs. Until now few microorganisms have been identified cerebrospinal fluid analysis (increase in protein concentration,
and associated with GBS: Campylobacter jejuni, Cytomegalovi- increase in mononuclear leukocytes count that does not
rus, Mycoplasma pneumoniae, Epstein-Barr virus, Haemophilus exceed 10 cells in 1 mm3), electrophysiological study (decrease
influenzae.7 It is believed that the triggering factor responsi- of conduction velocity in motor and sensory fibers, as well as
ble for an infection can be identified in 25%.8 Currently significant prolongation of distal latencies, and presence of
relation between presence of antibodies against ganglio- conduction block – informative about demyelinating nerve
sides and preceding C. jejuni infection, as well as theory that damage).
those antibodies cross-react with host's gangliosides is GBS should be differentiated from other diseases and
doubtless.9 disturbances causing acute muscle weakness e.g. myasthenia,
In 1976 in USA an increase in incidence of GBS has been periodic paralysis, myelitis transversa, poliomyelitis, brain-
reported after vaccination against influenza virus.10 Moreover stem inflammation, porphyrias and other neuropathies.
new cases were described after use of general anesthesia, after
delivery, or surgical procedures, as well as other factors.11,12 It 3.3. Treatment
has to be emphasized that GBS is not associated with genetic
inheritance and in 30% of cases no specific triggering factor is Each patient, suspected of having GBS should be hospitalized
established. because of highly variable character of the disorder, as well as
160 polish annals of medicine 21 (2014) 158–161

lack of possibility to predict symptoms intensity and their constantly modified, and changes ought to be preceded by a
progress. Extent and method of treatment highly depend on thorough evaluation of patient's health status.
patient's clinical status and phase of the disease. Patients who
experience a rapid increase in symptoms' intensity require 4. Conclusions
treatment in intensive therapy department with constant
monitoring of basic life functions with special attention to
respiratory and heart function. Plasmapheresis is considered 1. Diagnosis and treatment of GBS are important issues in
as primary treatment and its effectiveness has been con- clinical practice, because as much as 25% of patients can
firmed in few clinical studies.20 It is an invasive method of develop respiratory insufficiency.
treatment, used in specialized centers, only in cases of 2. Significant disability following remission of GBS is present
precise clinical recommendations, that include patients in approx. 20% of patients, and chronic fatigue is associated
fulfilling at least one of the following criteria: respiratory with 60%–70% of patients.
failure, inability to move independently, bulbar palsy.4 3. In spite of significant progress in treatment, GBS mortality
Usually three to five plasmapheresis sessions are carried accounts for 4%–15% and this is why early diagnosis,
out every second day and during one procedure about 50 mL appropriate treatment and prophylaxis of complications are
of blood plasma per 1 kg of body weight is being exchanged. of great importance.
While the patient undergoes treatment it is essential to
monitor blood pressure, heart's electrical activity (ECG), and
follow complete blood count before and after the procedure.
Conflict of interest
Plasmapheresis is not performed in patients who show ability
to walk independently.20,21 Treatment has to be initiated
during the first two weeks since the onset of disease. None declared.
Intravenous therapy with immunoglobulins given as 0.4 g/kg
dose over five following days is equally effective in treatment of
severe cases of GBS.22,23 Connection of both methods does not references
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