Guillian-Barr e Syndrome - A Case Study

Accident and Emergency Nursing ( 2002) 10, 92102
C 2002 published by Elsevier Science Ltd

doi:10.1054/yaaen.2001.0344, available online at http://www.idealibrary.com on
Guillian-Barre Syndrome
a case study
C. E. Toft
Acute Guillian-Barre Syndrome is an acute inflammatory demyelinating disease of the

peripheral nerves (Pfister & Bullas 1990) which affects the normal transmission of electrical
impulses along these nerves and consequently the function of the organs and tissues which
they innervate (Springhouse 1998, Waldock 1995). This disorder can rapidly replace an
individuals busy and active lifestyle with one of total dependence, often lasting months
(Waldock 1995). It is important, therefore, that nurses understand the pathophysiology of
the disease and its effect on the organs and tissues within the body, to enable them to
provide a high standard of care for patients suffering from this condition. This discussion of
Guillian-Barre Syndrome (GBS) will be in relation to a patient (who shall be called Jane Smith
for the purpose of this discussion) who was admitted to the Accident and Emergency (A&E)
department and diagnosed with GBS (see Box 1 for patient history). Within this discussion
GBS will be defined and its pathophysiology explained. The epidemiology and aetiology of
the disease will also be highlighted. The majority of the discussion will focus on the
physiological effects of GBS on the components of the peripheral nervous system and the
appropriate assessment and treatment measures. Finally, the outcomes of the disease will be
highlighted. The focus will be on the management of this condition within the A&E
department.
Introduction
Cheryl E. Toft
R. N. (Adult)
Bachelor of Nursing
(Hons), Accident &
Emergency
Department,
St Marys Hospital,
Paddington,
London, UK.
Tel.: 0207 886 6401;
E-mail: Cheryl Toft
@hotmail.com
GBS is an acute, inflammatory post-infectious

form of polyneuritis which comes out of the
blue (Worsham 2000). It is rapidly progressive
and can be potentially fatal if early diagnosis is
not made and appropriate interventions
commenced (Springhouse 1998, Hund et al.
1993, Waldock 1995). It is a generalized, usually
symmetrical, disorder affecting a number of
nerves at the same time but to a varying
degree. It results in motor weakness, sensory
abnormalities and, in some cases, autonomic
dysfunction (Worsham 2000). The outcome of
GBS can range from paraesthesia/weakness of
the lower limbs to the complete paralysis of all
voluntary muscles. Death can result from
respiratory muscle paralysis or cardiac
arrhythmias (McMahon-Parkes & Cornock
92 Accident and Emergency Nursing ( 2002) 1 0 , 9 2 1 0 2
1997). However, because GBS has not been

found to affect the central nervous system,
level of consciousness or cerebral function is
not affected.
Symptoms can develop over 1014 days
with the nadir usually being reached by the
third or fourth week. However, the progress of
this disease is variable and total paralysis and
respiratory arrest can occur within 48 hours
(McMahon-Parkes & Cornock 1997). The
progress of GBS follows three stages: the acute
phase, the plateau phase and finally the
recovery phase (see Table 1). It is during the
acute phase that patients usually seek medical
attention. The most common pattern to GBS is
the ascending version in which symptoms
affect the lower limbs first and progress
upwards towards the arms, trunk and cranial
nerves. This is the pattern that Jane Smith
Guillian-Barre Syndrome a case study
Table 1 The Stages of Guillian-Barre Syndrome

(Springhouse 1998, Pemberton 1998, Waldock 1995)
Box 1 Patient history

22-year-old Jane Smith presented to the
accident and emergency department
complaining of an onset of numbness and
tingling in her feet and fingers 2 days prior to
her admission. She described this as like
walking on cotton wool. Jane had also
experienced a gradual onset of weakness in her
feet, which had ascended to her lower legs
making walking increasingly difficult for her.
She had also experienced weakness in her
fingers making finer movements, such as
gripping a pen, difficult. Jane had no past
medical history but had suffered from a
gastrointestinal infection 3 weeks prior to the
onset of these symptoms.
Upon initial examination, the weakness in
Janes legs was symmetrical and severe. She
was unable to stand, let alone walk. When lying
on the trolley Jane appeared to have no control
over her legs and they just flopped to one side.
The weakness in her fingers had spread to her
hands making it difficult for her to grip
anything. However she was still able to raise
her arms and bend them at the elbow. Jane did
not appear to have any breathing difficulties or
speech/swallowing deficits. However her ankle
and knee tendon reflexes were absent.
Based on this clinical presentation Jane was
diagnosed as having Guillian-Barre syndrome.
She was moved to the resuscitation area where
her respiratory and cardiovascular function
could be monitored more closely.
Onset of first symptoms to the

time when no further
symptoms or deterioration
occurs
Usually lasts between 13 weeks
Can last up to six weeks in some
cases
The Plateau Phase
When the symptoms remain but

do not get any worse
Lasts from several days to 3 weeks
The Recovery Phase
Believed to coincide with

remyelination and axonal
process regrowth
Full recovery can take between 4
months and 3 years depending
on the severity of the condition
Recovery may never be complete
showed upon her admission to the A&E

department. However, other versions of GBS
can occur (see Table 2).
GBS affects around 0.52 individuals per
100 000 per year (Hund et al. 1993, Alter 1990).
However, this figure may be inaccurate due to
the milder forms of the condition being
undiagnosed. This condition can affect both
sexes but appears to be more predominant in
men (Alter 1990, McMahon-Parkes & Cornock
1997). McCleod et al. (1995) and Giberman
Table 2 Patterns of Guillian-Barre Syndrome (McCleod
et al. 1995, Griswold et al. 1984)
Common clinical pattern
Ascending GuillianBarre Syndrome
Throughout Janes stay in the department the

weakness progressed up her body until her legs
became completely paralysed and she was
unable to sit up by herself. However, although
the weakness in her hands worsened, it did not
progress up her arms. Janes consciousness
level did not deteriorate and she remained alert
and orientated throughout her stay in the
department.
Jane was eventually transferred to a specialized
neurological intensive care unit where she was
intubated and artificially ventilated due to her
reduced vital capacity and inability to cough.
The Acute Phase
Descending GuillianBarre Syndrome
Pure Motor GuillianBarre Syndrome
Pure Sensory
Guillian-Barre
Syndrome
Sensory and motor symptoms

begin in lower extremities
and progress upwards to
include the trunk, arms and
cranial nerves
Mild sensory symptoms Motor
symptoms more predominant
Weakness begins in the face or
bulbar muscles
Progresses downwards to
involve the limbs
Can quickly involve respiratory
function
Identical to ascending GBS but
with an absence of sensory
symptoms
Musculoskeletal pain is
generally not a problem
Predominant sensory signs and
symptoms
Absence of motor involvement
Accident and Emergency Nursing ( 2 0 0 2 ) 1 0 , 9 2 1 0 2 93
(1994) maintain that the incidence of this

condition peaks in the 1625 age group.
However, this is contradicted by Waldock
(1995) and Pemberton (1998) who believe that
adults over 40 are more likely to present with
this condition. Therefore it may be more
appropriate to highlight that this disorder can
affect all ages but has a higher incidence rate in
these age groups in particular.
GBS affects the nerves of the peripheral
nervous system which is composed of
structures which lie outside the brainstem and
spinal cord and includes the spinal, cranial and
autonomic nerves. It is responsible for carrying
the nerve impulses to and from the central
nervous system and has two functional parts,
the sensory division and the motor division,
which are subsequently divided into the
somatic nervous system (which supplies the
skeletal muscles) and the autonomic nervous
system (which supplies the smooth muscle,
cardiac muscle and glands) (Alexander et al.
1994, Tortora & Grabowski 1993).
In GBS, through a pathological process of
segmental demyelination, the myelin coat of
the peripheral nerves is destroyed and oedema
and inflammation of the nerve fibres occurs.
This myelin sheath usually acts as an electrical
insulator and protector. It also allows action
potentials to be delivered to the effector organ
or tissue via a process called salutatory
conduction. This process enables action
potentials to jump between the nodes of
ranvier without having to travel along the
whole length of the axon membrane and
consequently dramatically increases the
velocity of impulse conduction along the axon
(Tortora & Grabowski 1993, McCleod et al.
1995). Demyelination of a nerve means that the
action potential is unable to bridge the gaps
between what were the nodes of ranvier. Two
physiological consequences occur which affect
the nerves efficiency and consequently the
function of the organs/tissues which they
innervate. These are a reduction in the speed of
impulse conduction and conduction block. The
speed of conduction is reduced either by
impulse conduction being continuous across
the demyelinated segments, as in unmyelinated
nerves, or conduction between the nodes of
ranvier is delayed. Conduction block results
from the complete failure of the nerve fibres to
94 Accident and Emergency Nursing ( 2 0 0 2 ) 1 0 , 9 2 1 0 2
transmit electrical impulses across the

demyelinated segments (McCleod et al. 1995).
The nerve impulses are, therefore, slower to or
unable to reach their target cells and trigger a
response. Consequently, motor and/or sensory
deficits as well as autonomic dysfunction
ensues (McMahon-Parkes & Cornock 1997).
Although segmental demyelination is
considered the primary pathology, some degree
of axonal damage and further reduction in
nerve transmission can occur in more severe
cases of GBS (Hickey 1992, Morgan 1991).
Despite advances in research, this condition
remains a mystery with its precise cause being
unknown. One theory is that it is an
autoimmune response in which peripheral
nerves are damaged. Usually the immune
system does not attack its own tissues but in
GBS this normal recognition process is altered
(Murray 1993, Pemberton 1998, Waldock 1995).
It is thought that lymphocytes infiltrate the
nerve cells and produce proteases which cause
the degeneration of the myelin and Schwann
cells. The subsequent debris is then
phagocytosed by macrophages and it is during
this process that secondary axonal damage can
occur (Winer 1994, Pemberton 1998, Lindsay
et al. 1991). This theory is supported by the
finding that there is a history of some form of
viral illness in the 23 weeks prior to the onset
of the symptoms in 50% of patients diagnosed
with GBS (Alter 1990, Hund et al. 1993). Also,
microscopic findings have shown lymphocytic
infiltration of the nerves as well as the
congregation of immunoglobulins along the
myelin sheaths of the peripheral nerves
(Waldock 1995, Lindsay et al. 1991). However,
although the evidence incriminating the
autoimmune response as the cause of GBS is
growing, it is far from complete and further
research is needed. Also, no specific antigen
has been found nor the mechanism in the
abnormal immune response recognized (Hund
et al. 1993).
Although demyelination in GBS is typically
widespread, its extent can vary greatly from
one individual to another (McMahon-Parkes &
Cornock 1997). The signs and symptoms and
the progression of the condition will depend on
which nerves are affected during the
demyelination process and the extent of the
damage to the nervous tissue. As the
pathophysiology suggests, the clinical

manifestations in GBS are dramatic and can be
categorized into sensory, motor and autonomic
symptoms (Murray 1993).
Case study
Jane Smith presented to the department c/o of a
2-day history of numbness and tingling in her
feet and hands. This paraesthesia in a
stocking-glove distribution is a common
presenting symptom in GBS (McMahon-Parkes
& Cornock 1997) and can progress in an
ascending pattern to include legs, arms and
trunk.
These sensory deficits correlate with the
involvement of the afferent (sensory) fibres of
the spinal nerves in the demyelination process
of GBS. These nerve fibres usually convey
sensory information from the sensory receptors
in the skin, sense organs and viscera to the
central nervous system for interpretation and
initiation of a motor response, e.g. contraction
of muscle (Alexander et al. 1994). As it is the
heavily myelinated sensory nerves that are
affected, patients can also experience a
decreased appreciation of vibration and
proprioception (Murray 1993, Parobeck et al.
1992, Worsham 2000). A proportion of patients
do lose sense of touch, temperature and pain,
however, because the thinly myelinated nerves
(which support these sensations) are less
involved in the demyelination process, this is a
less common problem. As in Janes case, these
sensory symptoms usually recede and motor
symptoms become more prominent as the
condition progresses (Springhouse 1998). This
predominant motor pattern correlates with the
fact that GBS affects the more heavily
myelinated nerves which are involved in motor
function (McMahon-Parkes & Cornock 1997).
However, more recently, a possible variant of
the normal GBS pattern, which exhibits a more
prominent sensory involvement, has been
described (Giberman 1994).
Assessment of Janes sensory deficits were
carried out at regular intervals (through the
assessment of touch, pin prick and two point
discriminator) to assess the progression of the
disease.
One of the main complications of this
sensory deficit, which was experienced by Jane,
is the alteration of pain sensation (Meythaler

1997). Jane experienced muscle tenderness and
joint pain. Burning of extremities and an
increased sensitivity to touch and pressure can
also occur (Worsham 2000, Waldock 1995). Pain
relief was an important supportive measure in
Janes management and was provided through
the administration of analgesia as well as the
application of hot and cold compresses. The
use of amitryptiline or carbemazepine, to
reduce neuropathic pain, has also been
recommended (Lindsay et al. 1991). However it
has been recommended that narcotic analgesia
should be avoided in these patients due to its
depressing effect on respiratory function
(Griswold et al. 1984). Whether this is based on
research or personal preference is open to
question. Pemberton (1998) recommends that
narcotic analgesia may be required if severe
pain is experienced.
Prior to admission, Jane had also
experienced weakness in her feet. Upon
examination it was found that the weakness
had ascended to her legs and her hands,
making it difficult for her to stand up and grip
anything. Throughout her stay in the
department this weakness gradually became
worse in her legs until she was completely
paralysed. She also became unable to sit up by
herself.
Motor symptoms, in GBS, range from
muscle weakness to paralysis and commence in
the same pattern as the sensory symptoms, in a
stocking-glove distribution. Weakness is
usually symmetrical and progresses in an
ascending pattern up the body to include the
legs, arms, trunk, and face (cranial nerves)
depending on which nerves are involved in the
demyelination process.
The effect on the skeletal muscles is a result
of the demyelination of the efferent (motor)
neurones which transmit impulses from the
central nervous system to the effector tissues
(e.g. muscles). Usually, motor function, in the
form of muscle contraction, is initiated when an
electrical impulse (action potential) is produced
and transmitted down the nerve fibre to the
neuromuscular junction. The impulse is then
transmitted across the neuromuscular junction,
to the muscle, via the release of transmitters
which attach to the membrane of the muscle
cells and produce an electrical impulse (action
potential) in the muscle fibres, causing them to

contract (Tortora & Grabowski 1993, Alexander
et al. 1994, Roberts 1998). Demyelination of
these motor nerve cells results in a delay in
conduction or loss of impulse conduction
altogether and consequently muscle weakness
or paralysis, respectively. Signals to the legs are
possibly the most vulnerable to interruption
due to the distance the impulses have to travel,
which is why symptoms appear in the
extremities first (Worsham 2000).
Because of the rapid progression of the
disease, muscle atrophy will not be seen upon
initial assessment (Morgan 1991, Springhouse
1998). However, this may be seen in the later
stages of the condition. Usually peripheral
axons transport neurotransmitters, enzymes,
nutrients, cellular organelles and other
materials through a process of axoplasmic flow.
However, when the nerve is damaged the
previously innervated muscle degenerates due
to the loss of these trophic factors arising from
the nerve (Giberman 1994).
Because the reflex arc, involved in deep
tendon reflexes, depends on peripheral nerve
involvement, regular examination of Janes
tendon reflexes was carried out in order to
assess the progress of the condition, identify
which nerves were involved in the
demyelination process and predict the future
progression of the condition (Waldock 1995).
These are simple reflexes involving only one
synapse which can occur at the level of the
spinal cord. Usually vibrating or stretching (by
tapping with a hammer) the muscle at these
reflex points stimulates the muscle spindle and
causes an afferent stimulus which traverses the
monosynaptic arc and produces a reflex
contraction (McCleod et al. 1995) (see Table 3).
Table 3 This table shows the tendon reflexes that can

be tested to determine the progression of
Guillian-Barre Syndrome (Lindsay et al. 1991)
Tendon reflex
Spinal cord level
Deltoid
Biceps
C5, C6
C5, C6
Brachioradialis
Triceps
Knee
Ankle
C5, C6
C6, C7, C8
L2, L3, L4
S1, S2
Nerve tested
Circumflex nerve
Musculocutaneous
nerve
Radial nerve
Radial nerve
Femoral nerve
Sciatic nerve
Regular checking of motor strength was also

carried out to assess the extent and progression
of muscle weakness. This was done, as
recommended in the literature, by asking Jane
to move various parts of her body
(McMahon-Parkes & Cornock 1997). However,
this proved difficult, as a universal grading
system was not available. Although the
neurological limb assessment was used, this
did not provide a comprehensive assessment of
the different parts of the limbs affected or the
involvement of the trunk or cranial nerves. This
highlights the need for the development of a
universal grading system to be used by nurses
caring for patients with GBS.
Again, Janes treatment was supportive and
aimed at preventing secondary complications.
The affect on the motor division of the
peripheral nervous system led to Jane being
immobile and consequently at risk of skin
breakdown, deep vein thrombosis (DVT),
pulmonary embolism (PE) and the
development of contracture and muscle
atrophy. Vigilant pressure area care was
provided with the main aim of keeping Jane
comfortable. The use of splints and passive
movement of the limbs as well as prophylaxis
against DVT and PE is also recommended in
the literature (Worsham 2000, Murray 1993).
Prevention of contractures and muscle atrophy
is of particular importance due to the fact that
the majority of GBS patients will recover
through the regeneration of myelin and
damaged nerves. Motor function will not be
regained if muscles have atrophied and been
replaced with connective tissue
(McMahon-Parkes & Cornock 1997).
Jane did not experience any symptoms
involving the cranial nerves despite the fact
that they are commonly affected in GBS.
Therefore the effect of GBS on the cranial
nerves will not be discussed except in relation
to respiratory and autonomic function (refer to
Table 4 for a summary of the demyelination
effect on cranial nerves).
A potentially fatal complication of GBS
occurs when demyelination spreads to and
affects the transmission of impulses in the
phrenic and intercostal nerves, which supply
the respiratory muscles. This can occur rapidly
and leads to ventilatory problems, eventual
respiratory failure and death (George 1988).
Table 4 This table shows the symptoms corresponding

with cranial nerve involvement in Guillian-Barre
Syndrome (Blows 2001, Murray 1993)
Cranial Nerves III,
IV, & VI
(Oculomotor,
Trochlear &
Abducens
Nerves)
Cranial Nerve V
(Trigeminal
nerve)
Drooping of upper eyelid (ptosis)

Double vision (diplopia)
Deviation of eyeball upwards and
outwards
Cranial Nerve VII

(Facial nerve)
Facial weakness/paralysis
Loss of smiling and frowining,
Difficulty closing eyes
Cranial Nerve IX
(Glossopharyngeal nerve)
Loss of taste
Difficulty swallowing
Cranial Nerve X
(Vagus nerve)
Autonomic dysfunction such as:

Cardiac arrhythmias
Hypotension/hypertension
Sinus tachycardia (loss of
parasympathetic inhibition)
Cranial Nerve XI
(Accessory
nerve)
Inability to shrug shoulders

Inability to rotate head
Cranial Nerve XII

(Hypoglossal
nerve)
Loss of taste
Difficulty swallowing
Speech deficits due to paralysis
of the tongue
Loss of sensation in face, forehead

and temple
Difficulty chewing
Respiratory muscles are affected in 25% of GBS

cases with 20% needing assisted ventilation
(Giberman 1994).
External respiration relies upon adequate
ventilation to transport oxygen to the alveoli, in
the lungs, where it can then diffuse into the
pulmonary capillaries along a pressure
gradient and be transported to the cells for
cellular metabolism. Ventilation, in turn, relies
upon the contraction of the intercostal muscles
and the diaphragm which are innervated by
the intercostals and phrenic nerves respectively.
Inspiration occurs through the contraction of
these muscles which enable the lungs to
expand. This increases the volume of the lungs
and creates an internal negative pressure
allowing air to be drawn into the lungs from
the atmosphere along a pressure gradient
(Dolan & Holt 2000, Tortora & Grabowski 1993,
Roberts 1998). Ventilation is also essential for
excreting carbon dioxide, the waste product of
cellular metabolism, through expiration.
In GBS, however, demyelination of these
respiratory nerves and consequent
abnormalities in innervation, leads to

intercostal and diaphragmatic muscle
weakness and fatigue and in severe cases
paralysis (George 1988, Pfister & Bullas 1990).
This impairment of respiratory muscles leads
to a reduced concentration of oxygen reaching
the alveoli and being diffused into the blood.
Consequently, the blood leaving the lungs has a
low concentration of oxygen leading to hypoxia
which in turn threatens homeostasis.
Ventilatory insufficiency also leads to the build
up of carbon dioxide in the blood. These
variations in arterial oxygen and carbon
dioxide concentrations as well as the blood pH
are detected by peripheral and central
chemoreceptors. Normally these receptors
stimulate the respiratory centre, via the vagal
and glossopharyngeal cranial nerves, to
increase the respiratory rate and depth so as to
restore normal oxygen and carbon dioxide
levels. This is achieved through the stimulation
of the diaphragm and intercostal muscles.
However, in GBS, this function is also impaired
by the demyelination of the cranial, phrenic
and intercostal nerves. Consequently, oxygen
and carbon dioxide levels are not returned to
normal. Low concentrations of oxygen can
eventually lead to hypoxia and the build up of
carbon dioxide leads to respiratory acidosis.
When tissues are not supplied with enough
oxygen to maintain metabolism and cannot
eliminate enough carbon dioxide to prevent
respiratory acidosis, respiratory failure ensues.
This leads to dysfunction in other organs and
can lead to circulatory collapse and cardiac
arrest (Tortora & Grabowski 1993, Roberts
1998).
Hypotonia of the respiratory muscle also
occurs as a result of muscle weakness and
fatigue. This results in smaller resting lung
volumes and a lower respiratory force being
generated. The use of the accessory muscles is
needed in order to maintain adequate
ventilation. However, this results in
increasing oxygen demand which in turn
exacerbates the problem of respiratory
insufficiency and alveolar hypoventilation
(George 1988).
Although Jane did not display any signs of
respiratory insufficiency upon initial
assessment, it was important to continuously
monitor her respiratory function in order to
assess respiratory deterioration and the need

for intubation and assisted ventilation.
A simple, but effective, measure, which was
used in Janes management to help reduce the
incidence of hypoxia and respiratory acidosis,
was the administration of oxygen therapy
through a re-breath mask. This increases the
concentration of oxygen in the lungs and helps
to set up a concentration gradient between the
alveoli and the blood allowing more oxygen to
diffuse into the blood and be transported to the
tissues (Dolan & Holt 2000). However, if there
is inadequate ventilation this may only succeed
in increasing the level of carbon dioxide levels.
Therefore the concentration of oxygen
administration should be kept as low as
possible while maintaining adequate blood gas
levels.
Arterial blood gases were taken regularly to
assess the effectiveness of respiration and
detect the development of hypoxia, respiratory
acidosis and impending respiratory failure
before clinical signs of respiratory insufficiency
became evident (Roberts 1998). A reduced
arterial oxygen level below 60 mmHg and a
carbon dioxide level above 50 mm Hg indicates
severe hypoxia and respiratory acidosis and
impending respiratory failure. Assisted
ventilation should be considered at this point
(Pfister & Bullas 1990; George 1988) (see Table 5
for normal blood gas levels).
Pulse oximetry was also used in Janes
management. This measures the amount of
haemoglobin saturated with oxygen (Dolan &
Holt 2000). However, although this is a useful
indicator of oxygen concentrations, it does not
alert the medical team to the amount of carbon
dioxide accumulating in the blood.
Measurement of Janes vital capacity was
also essential to assess the ability of the
respiratory muscles to expand the lungs and
allow air entry (Tortora & Grabowski 1993).
This was taken by using a spirometer which
measures the maximum amount of air that can
Table 5 Normal arterial blood gas levels (Dolan & Holt
2000)
PaCO2
PaO2
pH
Bicarbonate
4.86.1 KPa (3646 mmHg)

1013.3 KPa (75100 mmHg)
7.357.45
2428 mmol/L
be exhaled after maximum inhalation and

reflects the lung volume which can be
generated by the patient (George 1988). It is
important to remember that this measurement
may be difficult to obtain with facial weakness/
paralysis through the demyelination of cranial
nerve VII (the facial nerve). A vital capacity of
15 ml/kg of body weight should alert the
medical team to consider intubation and
artificial ventilation (Murray 1993, Parobeck
et al. 1992).
Regular assessments of Janes rate and depth
of breathing were carried out. Rapid shallow
breathing (30 breaths/minute) is indicative of
respiratory muscle fatigue and weakness and
increases the risk of hypoxia and respiratory
acidosis (George 1988, Hund et al. 1993,
McMahon-Parkes & Cornock 1997). Other
indicators of respiratory distress include
cyanosis, tachycardia, use of accessory muscles,
nasal flaring, chest wall retraction, anxiety,
restlessness and confusion (George 1988, Dolan
& Holt 2000).
Verbal communication with Jane enabled her
speech to be assessed. Speech deficits can be an
indicator of cranial nerve involvement.
Weakness in the tongue and throat muscles can
compromise the airway. Intubation to protect
the airway may be required.
Patients with GBS also have the added
complication of retention of secretions in the
lungs and oral cavity secondary to inadequate
mucociliary clearance. Demyelination of the
cranial nerves IX (glossopharyngeal), X (vagal)
and XII (hypoglossal) result in the ineffective
swallow or gag mechanisms which usually
prevent accumulation of secretions in the oral
cavity (George 1988). Coughing is a high
velocity manoeuvre achieved during
respiration by the combined work of the
diaphragm, intercostal, accessory and
abdominal muscles and helps to clear the
bronchial tree of secretions. The weakening of
the diaphragm and intercostal muscles may
reduce the patients ability to cough. The
impairment of these functions results in a build
up of secretions in the oral cavity and lungs
causing airway obstruction, further difficulty in
breathing and an increased likelihood of
pulmonary infection. (McMahon-Parkes &
Cornock 1997). Regular assessments of Janes
swallow, gag and coughing mechanisms were
also carried out to assess for respiratory

deterioration. Removal of secretions by
suctioning may be necessary in some cases.
A reduced vital capacity, abnormal arterial
blood gas levels and an inability to clear
secretions signals ventilatory failure and
should alert the medical team to the need for
intubation and assisted ventilation before
respiratory failure occurs (Pfister & Bullas
1990). Throughout Janes stay in the
department her respiratory function did
deteriorate and she was transferred to a
neurological intensive care unit for these
interventions.
The degree of autonomic dysfunction
resulting from GBS is variable and the
pathogenesis has not been clearly established
(Ferraro-Herrera et al. 1997). Theories include
peripheral sympathetic nerve demyelination,
abnormalities of sympathetic ganglia, lesions of
cranial nerve X (vagal nerve) at the medulla or
endocrine disturbances (Dalos et al. 1988). It is
most likely that a combination of factors play a
part in autonomic dysfunction (Parobeck et al.
1992). Autonomic dysfunction may lead to
cardiac arrythmias, tachycardia or bradycardia,
fluctuations in blood pressure, inappropriate
secretion of anti-diuretic hormone and bladder
and bowel dysfunction (Pemberton 1998). In
fact it has been found that cardiovascular
symptoms can occur in up to 65% of patients
with GBS (McMahon-Parkes & Cornock 1997,
Winer 1994).
The autonomic nervous system (ANS)
usually conveys sensory impulses from
receptors, located primarily in the viscera, to
the central nervous system. There, the
information is interrupted and an impulse
relayed by the autonomic motor neurons to the
involuntary tissues such as cardiac muscle,
smooth muscle and glandular tissue (Tortora &
Grabowski 1993). It consists of two divisions,
the sympathetic and parasympathetic, which
work antagonistically to maintain and quickly
restore homeostasis in the body. Any condition
which affects this autonomic nervous tissue
will consequently affect the efficient
coordination of these two divisions (Tortora &
Grabowski 1993, Roberts 1998). In particular,
demyelination may affect the afferent and
efferent limbs of the cardiovascular reflexes
and the baroreceptor reflexes (Lindsay et al.
1991) which in turn affect the function of the

heart and the regulation of blood pressure. This
is what the rest of this discussion will focus on.
Blood pressure is usually regulated by the
transmission of sensory information from
baroreceptors (which monitor stretching of
blood vessel walls) to the vasomotor centre of
the brain. Normally impulses are then relayed
along the efferent motor fibres, which run in
the vagus nerve to the heart and in the
sympathetic nerves to the heart, mesenteric
vascular bed and blood vessels in the skin and
muscles. Here, in conjunction with chemical
neurotransmitters, they can cause a change in
these tissues according to the needs of the body.
An increase in blood pressure will generate
impulses along the autonomic nerve fibres,
which cause a reduction in heart rate and
peripheral vasodilation. This reduces blood
pressure. Alternatively, a reduction in blood
pressure will generate impulses which increase
the heart rate and stimulate peripheral
vasoconstriction resulting in an increase in
blood pressure (Tortora & Grabowski 1993,
Alexander et al. 1994). Impairment of
autonomic nerves disrupts this regulatory
function and causes the fluctuating blood
pressures seen in GBS. Neuro-hormonal control
of blood pressure through catecholamines,
atrial natriuretic factor and anti-diuretic
hormone can also be altered through the
impairment of nerve impulse conduction.
However, the process by which this occurs is
little understood (Murray 1993).
Postural hypotension is another
complication of GBS resulting from the
impairment of nervous control of the
splanchnic vascular bed. On standing, 700 ml
of blood leaves the chest and pools in the legs.
Normally vasoconstriction of the splanchnic
vascular bed and blood vessels in the skeletal
muscle causes the redistribution of blood so as
to maintain normal blood pressure (McCleod et
al 1995).
Autonomic regulation of cardiac muscle is
also impaired in GBS. Although the cardiac
muscle has the intrinsic ability to generate a
contraction stimulus through its built-in
pacemaker, the sino-atrial node (SA node), the
autonomic nervous system normally plays a
role in the regulation of this pacemaker. It can
alter the rate of impulse generation by the SA
node, the speed of impulse conduction and

strength of cardiac contraction through both
sympathetic and parasympathetic pathways.
The sympathetic nerve fibres increase the heart
rate, conduction speed through the
atrioventricular node (AV node) and the force
of contraction. However, the parasympathetic
system, via the vagus nerve, reduces heart
rate, rate of conduction through the AV node
and the force of contraction. These pathways
alter the contraction of cardiac muscle
according to the changing needs of the body
which are detected by baroreceptors and
chemoreceptors (Alexander et al. 1994, Roberts
1998). A disturbance in the electrical impulses
transmitted to the pacemaker will consequently
affect the electrical impulses produced by the
pacemaker which control the rate and rhythm
of the heartbeat. As a result cardiac arrhythmias
are commonly produced in GBS which can lead
to cardiac arrest if the patient is not closely
monitored and timely interventions made.
Jane did not show any signs of autonomic
abnormalities throughout her stay in the
accident and emergency department. However,
because autonomic dysfunction is a common
cause of death, vigilant cardiovascular
monitoring was employed. This was through
continuous cardiac monitoring and regular
blood pressure measurements. It has been
recommended that autonomic dysfunction
should be treated conservatively, without drug
therapy, since its underlying cause is neural
disruption (Dalos et al 1988). However, surely if
a fatal outcome is possible from cardiovascular
abnormalities drug therapy should be
considered. Ferraro-Herrera et al. (1997)
suggest that antihypertensives be used in
hypertension. However, these should be
administered with caution due to the
possibility of fluctuating an already unstable
blood pressure.
Diagnosis of GBS, during initial
presentation, is based on clinical manifestation
(see Table 6). Certain diagnostic interventions
can be completed to support this diagnosis.
However, because the results of these tests are
not significant during the initial stage, they are
not usually carried out until later on in the
progression of the condition (Winer 1994).
There is no cure for GBS and its treatment is
supportive in nature through the monitoring of
100
Accident and Emergency Nursing ( 2 0 0 2 ) 1 0 , 9 2 1 0 2
Table 6 Major diagnosis criteria for Guillian-Barre

Syndrome (McMahon-Parkes & Cornock 1997)
Motor
Progressive weakness of the skeletal

muscles, generally ascending and
symmetrical
Reduction or loss of deep tendon
reflexes
Reduced pulmonary capacity
Weakness of facial muscles and
dysphagia
Sensory
Parasthesia in extremities
Reduced proprioception
Sensory loss occurs in a stocking-glove
distribution
Hypertension, hypotension or labile
blood pressure
Tachycardia or bradycardia
Cardiac arrhythmias
Autonomic
the progression of the condition, the

management of symptoms and the prevention
of complications. However, some therapies,
which claim to improve the recovery of GBS
patients, are discussed within the literature.
Discussion of these investigations and
treatment therapies is outside the realms of this
discussion which focuses on the initial
management of GBS (see Tables 7 & 8).
Overall, the outcome of GBS is usually very
good, with 80% of patients making a full
recovery (Pemberton 1998, McMahon-Parkes &
Cornock 1997). However, in 5% of cases, GBS
can be fatal and this mortality rises to 1530%
Table 7 There is no specific test that can confirm the
diagnosis of Guillian-Barre Syndrome. However, several
diagnostic tests can be used to support the clinical
presentation as shown below (Springhouse 1998,
Worsham 2000, McMahon-Parkes & Cornock 1997)
Cerebrospinal
Fluid (CSF)
Electromyography
Nerve Conduction
Studies
Obtained via a lumbar puncture

Shows an elevated protein
level but no rise in white
cell count. This is known
as Albuminocytologic
Dissociation
Rise in protein level does
not occur until 12 weeks
after the onset of symptoms
Rise in CSF pressure
Shows reduced electrical activity
in the Muscles
Shows activation of single
muscle cells instead of
Activation of groups of
muscle fibres
Shows a reduction in nerve
conduction velocity
Table 8 Treatment of Guillian-Barre Syndrome (Winer 1994, Springhouse 1998, Meythaler 1997, McMahon-Parkes
& Cornock 1997)
Corticosteroid therapy
Used in the 1960s due to the belief that it would suppress the
autoimmune response in Guillian-Barre Syndrome
Controlled studies by Hughes et al. (1978) and the Guillian-Barre
Syndrome Steroid Trial Group (1993) found steroids to be
ineffective in speeding the recovery of patients
Plasmapheresis
Form of dialysis where the patients blood is filtered to remove

circulating anti-myelin antibodies
More effective if commenced within the first few days of the onset of
symptoms
Claims to increase the speed of recovery, shorten the duration of
muscle weakness, prevent/reduce the need for ventilatory
assistance and reduce long-term disability
Research is contradictory with some studies reporting the benefits of
plasma exchange whilst others report no significant improvement
Therapy is uncomfortable, potentially dangerous and expensive and
so is reserved for the most severely affected or whose illness is
rapidly progressing
Equally effective as plasmapheresis
Exact mode of action is unknown
? binds antibodies to receptor sites on the nerve cells to prevent the
attachment of anti-myelin antibodies
Little research-based evidence to back up the benefits of the therapy
High incidence of relapse
Intravenous immunoglobulin (IVG)
in those patients requiring assisted ventilation

(Hund et al. 1993). The majority of the
morbidity and mortality in this condition has
been related to the secondary complications of
the disease such as pulmonary infections,
sepsis, cardiac arrhythmias, cardiac arrest,
pulmonary emboli and delayed treatment of
respiratory failure (Meythaler 1997). This
highlights the need for vigilant assessment and
supportive management. It has been found that
some degree of permanent motor or sensory
deficits (in the form of minor weakness,
numbness and discomfort) can remain in 15%
of patients and this can be severe in 5% of
patients (Hund et al. 1993).
Recovery usually occurs in a descending
pattern with patients regaining muscle strength
in the opposite direction to which it was
lost. The length and degree of recovery varies
among patients and is related to the extent of
neuronal damage that has occurred as well as
muscle atrophy. The ability to recover from this
condition is due to the fact that Schwann cells
are able to mitose and remyelinate the nerves
(Tortora & Grabowski 1993, McMahon-Parkes
& Cornock 1997). Remyelination can be a
fast process taking between 24 weeks but full
recovery can take up to 6 months or even 3 years
in some of the more severe cases (Pemberton
1998, McCleod et al. 1995). Poorer prognosis

and prolonged recovery has been related
to the presence of axonal damage, respiratory
failure and a long delay between the time
of maximum paralysis and the commencement
of recovery (Lindsay et al. 1991).
Conclusion
This discussion of GBS has focused on the
pathophysiology of the condition and the effect
that the demyelination of the peripheral nerves,
has on the sensory, motor and autonomic
functions of the body. This condition has the
potential to result in death through the
paralysis of the respiratory muscles and
autonomic disruption leading to cardiovascular
complications. GBS remains a mystery with an
unknown aetiology and no cure. Therefore
treatment remains supportive, through the
monitoring of deterioration, control of
symptoms and prevention of complications.
Nurses should be aware of the pathophysiology
of this condition and the effect that it has on the
organs and tissues within the body so as to be
able to provide a high standard of care and
ensure that timely, life-saving interventions are
initiated. Possibly the most challenging aspect
of nursing care is providing patients, who have
gone from a life of independence to complete

dependence on nursing care within days, and
their families, with the emotional, social and
psychological care that they require.
Unfortunately, until a cause and cure is
found, this will remain a devastating and
sometimes fatal condition requiring intensive
care and the skills of a dedicated nursing and
medical team.
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Guillian-Barr e Syndrome - A Case Study

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Accident and Emergency Nursing ( 2002) 10, 92102

C 2002 published by Elsevier Science Ltd

Acute Guillian-Barre Syndrome is an acute inflammatory demyelinating disease of the

GBS is an acute, inflammatory post-infectious

92 Accident and Emergency Nursing ( 2002) 1 0 , 9 2 1 0 2

1997). However, because GBS has not been

C 2002 published by Elsevier Science Ltd