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Guillain-Barr syndrome

Betsy Hellewell

PSYC 1100

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What is Guillain-Barr syndrome?

Guillain-Barr syndrome can affect any age both male and female. It is rare

and only affects one person in 100,000. Guillain-Barr syndrome is a disorder

where the body's immune system attacks part of the peripheral nervous system.

People that get Guillain-Barr syndrome usually have rapid loss of mobility

in their upper and lower extremities. In August 2009 my husband was diagnosed

with this syndrome. In 7 days after the first symptom he could not walk or hold

onto things with his hands. We found out that there is no cure but recovery is

possible. High doses of immunoglobulin therapy the best treatment we have for

now along with physical and occupational therapy

The symptoms start as weakness in the legs and then the arms. The weakness

continues working inward and upward until the person can no longer walk and

loses the ability to use their hands and arms. The symptoms can progress as rapid as

in hours, days or weeks. The height of weakness usually occurs in two weeks. If left

untreated the muscles in the body become more and more weak until almost

paralyzed. In these cases, the disorder is life-threatening and is considered a

medical emergency. The individual is often put on a ventilator to assist with

breathing. Most people recover from Guillain-Barr syndrome, but about 30

percent continue to have some weakness after 3 years. Around 3 percent may suffer

a relapse of muscle weakness and tingling sensations many years after the initial

attack.

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The cause of Guillain-Barr syndrome is not known. What scientists do

know is that the body usually has a virus. The immune system that fights off

infection does their job and then dont stop. After the immune system kills off the

virus it looks for something to attack and starts on the myelin sheath that surrounds

the axons in the nervous system. This is why a person with Guillain-

Barr syndrome has weakness in their legs and arms first because the signals to

these parts of the body have the longest distance to travel.

Guillain-Barr syndrome can be difficult to diagnose. It has symptoms

similar to other disorders. My husband was tested for stroke or heart attack. As

doctors question patients they look for how quickly the symptoms progressed and

the order and symmetry of the progression. This will allow them to see the

difference from other disorders. Testing reflexes is another test given to help in the

diagnosis. Reflexes such as knee jerks are usually lost. Because the signals traveling

along the nerve are slower, a nerve conduction velocity (NCV) test can give a doctor

clues to aid the diagnosis.

There is no known treatment for Guillain-Barr syndrome, but therapies can

lesson the severity of the illness and accelerate the recovery in most patients. After

treatment and therapy my husband is not cured but has recovered 90 percent.

Plasmapheresis (also known as plasma exchange) and high doses of immunoglobulin

therapy are used to reduce the severity and duration of Guillain-Barr episode.

Investigators have found that giving high doses of these immunoglobulins, derived

from a pool of thousands of normal donors to Guillain-Barr patients can lessen the

immune attack.

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Scientists are looking to find new treatments and making the existing ones

better. Scientists are also looking at the workings of the immune system to fine the

cells that are responsible for beginning and carrying out the attack on the nervous

system. They are looking at the correlation that viral or bacterial infections have on

Guillain-Barr syndrome.

Because of the treatment and therapy my husband received, and time, he

now has full mobility with his arms and hands. His legs have recovered but he

cannot feel from his ankles down. He suffers a relapse of muscle weakness and

tingling sensations when he is over fatigued and tired. We know that the cause of

Guillain-Barr syndrome is still not known. We also know that scientists are

looking to find new treatments. For now plasmapheresis and high doses of

immunoglobulin therapy along with physical and occupational therapy is the best

course to take.

4
National Institute of Neurological Disorder and Stroke,

https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-

Sheets/Guillain-Barr%C3%A9-Syndrome-Fact-Sheet, July 25, 2017

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http://jamanetwork.com/journals/jamaneurology/fullarticle/7
89059
Neurological Review
August 2005

Supportive Care for Patients With


Guillain-Barr Syndrome
Richard A. C. Hughes, MD; Eelco F. M. Wijdicks, MD; Estelle Benson; et alDavid R.
Cornblath, MD; Angelika F. Hahn, MD; Jay M. Meythaler, MD; John T. Sladky, MD; Richard
J. Barohn, MD; James C. Stevens, MD
Author Affiliations Article Information
Arch Neurol. 2005;62(8):1194-1198. doi:10.1001/archneur.62.8.1194

Abstract
A multidisciplinary consensus group searched MEDLINE from 1966 to May 2003,
extracted relevant references, and prepared recommendations on supportive
care for Guillain-Barr syndrome. In the absence of randomized controlled trials,
we agreed on recommendations by consensus based on observational studies
and expert opinion. In the acute phase in bed-bound adult patients, the group
recommended the use of heparin and graduated pressure stockings to prevent
deep vein thrombosis, monitoring for blood pressure, pulse, autonomic
disturbances, and respiratory failure, and the timely institution of artificial
ventilation and tracheostomy. Pain management is difficult, but carbamazepine or
gabapentin may help. The cautious use of narcotic analgesics may be needed.
Disabled patients should be treated by a multidisciplinary rehabilitation team and
should receive an assistive exercise program. Persistent fatigue following
Guillain-Barr syndrome is common and may be helped by an exercise program.
Because of a very small and possibly only theoretical increase in the risk of
recurrence following immunization, the need for immunization should be reviewed
on an individual basis. More research is needed to identify optimal methods for
all aspects of supportive care.

A recent practice parameter recommended either intravenous immunoglobulin or


plasma exchange, but not corticosteroids, as appropriate treatments for adults
and probably children with severe Guillain-Barr syndrome (GBS) within 2 weeks
from onset.1Most patients in the trials of these treatments have had the
demyelinating form of the disease, and the benefits of treatment in uncommon
subgroups, such as those with axonal disease, cannot be distinguished. Despite
immunotherapy, 4% to 15% of patients with GBS die from this syndrome2- 6 and
nearly 20% have a persistent disability.5 Death from GBS occurs mostly in
mechanically ventilated patients. Supportive care remains the mainstay of
treatment, but the evidence for the methods of supportive care is inadequate and
consensus guidelines for treatment have not been published.

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Evidence review
The consensus group of 6 neurologists with a special interest in GBS, 1 physical
medicine specialist, 1 evidence-based medicine specialist, and 1 patient
advocate met and decided which questions to review.

We searched MEDLINE from 1966 onward (last search in May 2003) for articles
including the term polyradiculoneuritis, limited by human and cross-referenced
with the terms therapy, pain, nutrition, diet, pulmonary
ventilation, tracheostomy, artificial respiration, autonomic nervous
system, dysautonomia, arrhythmia, neurogenic
bladder, urinaryincontinence, urination disorders, constipation, physical
therapy, occupational therapy, palliative care, supportive
care, thrombophlebitis, immunization, and recurrence. We also searched the
Cochrane Library register of randomized trials (issue 3, 2003) with Guillain-Barr
syndrome as the search term. Two members of the group prepared draft
statements and recommendations that were circulated through the entire group
repeatedly until consensus was achieved. Our recommendations are, therefore,
consensus statements based on observational studies of GBS, inferences from
randomized controlled trials in other conditions, and expert opinion.

Prophylaxis for deep vein thrombosis


Immobilization owing to GBS is a risk factor for the development of deep vein
thrombosis (DVT).7 Time to developing DVT or pulmonary embolus varies from 4
to 67 days after onset.7,8 Children have a very low incidence of DVT.9 There is a
lack of clinical studies that address methods of prophylaxis against thrombosis in
GBS, duration of prophylaxis, or monitoring of patients at risk for thrombosis.
Observational studies in orthopedic or general surgery patients suggest a benefit
from subcutaneous heparin (5000 U, 12-hourly) in preventing DVT.9 In acutely ill
medical patients, prophylactic treatment with subcutaneous enoxaparin (40 mg
daily) reduced the incidence of DVT from 15% in the placebo group to
approximately 5% in the treated patients.10 In a recent meta-analysis, support
stockings reduced the risk by almost 70% in patients at moderate risk for
development of postoperative thromboembolism.11

Recommendation
Subcutaneous unfractionated or fractionated heparin and support stockings are
recommended for nonambulant adult patients until they become able to walk
independently.

Cardiac and hemodynamic monitoring


Serious and potentially fatal disturbances of autonomic function, including
arrhythmias and extreme hypertension or hypotension, occur in approximately
20% of patients with GBS.12,13 Severe bradycardia may be preceded by wide

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swings, exceeding 85 mm Hg, of systolic blood pressure from day to
day.14 Bradycardia may be so severe as to cause asystole, which may require a
cardiac pacemaker.12 Endotracheal suction or pharmaceutical agents may
provoke these changes.14,15 Other significant autonomic disturbances in GBS are
adynamic ileus, hyponatremia, and deficiencies in bronchial mucosal function.
Most, but not all, dysautonomic complications occur among patients with
advanced generalized weakness and respiratory failure.16 In 1 prospective
study,17 a reduction in beat-to-beat variation in heart rate predicted subsequent
dysautonomia.

Recommendation
Monitoring of pulse and blood pressure is recommended in patients who are
becoming severely affected by GBS until they have discontinued the ventilatory
support and have had their tracheostomy removed or until they have begun to
recover without needing either intervention. The evidence concerning the method
and setting of monitoring is insufficient to make specific recommendations.

Respiratory monitoring and airway protection


Neuromuscular respiratory function becomes compromised in 17% to 30% of
patients with GBS.6,16,18- 21 In some patients, bulbar dysfunction causes difficulty
with clearing secretions, compromising gas exchange and increasing the risk of
aspiration.22- 24Clinical features that indicate fatigue of respiratory muscles are
tachypnea, sweating, tachycardia, asynchronous movements of the chest and
abdomen, and episodic use of accessory muscles of respiration. In 1 case
series,22 rapid disease progression, presence of bilateral facial palsy, and
autonomic dysfunction increased the likelihood of intubation. A study of 722
patients, of whom 313 required ventilation, identified 6 predictors of the need for
ventilation in a multivariate analysis: time from onset to admission being less
than 7 days, inability to cough, inability to stand, inability to flex the arms or head,
and liver enzyme level increases.25 In 196 of the patients with available vital
capacity measurements, predictors were time from onset to admission being less
than 7 days, inability to lift the head, and vital capacity of less than 60% of that
which was predicted. In many patients, a vital capacity (measured volume with
forceful exhalation after maximal inhalation) below 20 mL/kg, a PImax (maximum
inspiratory pressure generated after maximal sucking in through a mouth piece
while occluding the nose) of less than 30 cm H2O or a PEmax (maximum
expiratory pressure generated on maximal blowing out) of less than 40 cm H2O
warns of imminent respiratory arrest.26- 28 Rapid decline in vital capacity (eg, 50%
from baseline) may further indicate impending respiratory failure, but this finding
needs confirmation.26,27 Patients with pulmonary infiltrates or atelectasis
generally require intubation and mechanical ventilation. Hypoxemia is also an
indicator of neuromuscular respiratory failure. Hypercarbia appears later.21 Thus,
respiratory failure in GBS is common and life threatening. Emerging
diaphragmatic failure can be detected by serial clinical observation or respiratory

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function tests. Patients who require ventilation are at high risk of suffering major
complications including pneumonia, sepsis, gastrointestinal tract bleeding,
pulmonary embolus, and others. In a series of 114 patients, 60% admitted to an
intensive care unit had major complications in 1 of these categories.29

Recommendation
Respiratory function should be monitored in patients with GBS, but there is
insufficient evidence to recommend specific methods. Weaning from the
ventilator should be guided by improvement in strength and serial pulmonary
function tests.

Timing and method of tracheostomy


The mean duration of ventilation in various treatment trials has ranged between
15 and 43 days, suggesting that a proportion of patients can be spared from
receiving a tracheostomy.28,30- 32 Early tracheostomy increases patient comfort
and airway safety and may help weaning. On the other hand, surgical
tracheostomy results in permanent disfigurement and has sometimes been
associated with life-threatening hemorrhage, infection, accidental dislodgement
of the tube, fatal procedure-related necrotizing mediastinitis, chyle fistula due to a
thoracic duct perforation, and a cosmetically unacceptable, hypertrophic keloid
tracheostomy scar. More recently, percutaneous dilatational tracheostomy has
been introduced, but this technique has not been compared with traditional
tracheostomy in GBS. In a randomized trial in patients selected for elective
tracheostomy, percutaneous dilatational tracheostomy was superior.33 The
procedure involves a small skin incision and then insertion of a cannula into the
trachea, followed by dilators of gradually increasing size until the desired
tracheostomy tube can be accommodated. Percutaneous tracheostomy may
reduce the risk of accidental extubation owing to the fact that it fits more snugly
around the stoma. A better cosmetic outcome may result from a smaller skin
incision.34- 36

A newly introduced pulmonary function ratio has been used to predict the need
for tracheostomy.37 Daily vital capacity and maximal inspiratory and expiratory
pressures were summed to create an integrated pulmonary function score. A
pulmonary function ratio was calculated, which represents the pulmonary function
score at day 12 after intubation divided by the pulmonary function score at the
day of intubation. This study found that at day 12 with a pulmonary function ratio
of less than 1, it is highly unlikely that patients will be weaned from the ventilator
within 3 weeks and tracheostomy should be performed. The sensitivity of a
pulmonary function ratio of less than 1 for predicting that the duration of
ventilation would be more than 3 weeks was 70%, and the specificity and positive
predictive value were 100%.

Recommendation

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The decision to place a tracheostomy may be postponed for 2 weeks. If after 2
weeks the pulmonary function tests do not show any significant improvement
from baseline, tracheostomy should be performed. If the pulmonary function test
tends to improve above baseline, tracheostomy could be deferred for an
additional week, allowing the patient to attempt to be weaned from the ventilator.
Percutaneous tracheostomy may be preferred in centers with adequate
experience in using the technique.

Pain management
Retrospective observational analyses of GBS case series have documented pain
as an early symptom, with an incidence ranging from 33% to 71%.38- 43 One
study examined incidence and intensity of pain prospectively and quantified the
response to medical pain intervention.41 Pain was reported by 89% of the
patients and was severe in half. First-line drugs that were used were
acetaminophen and nonsteroidal anti-inflammatory drugs. However, 75% of the
patients additionally required oral or parenteral opioids and 30% of the patients
were treated with intravenous morphine infusions (range, 1-7 mg/h). Ten percent
of the patients received tricyclic antidepressants and a further 10% received
carbamazepine as adjuvant treatments for neuropathic pain during the later
course of the illness. In a randomized, double-blind, crossover trial involving 18
participants, gabapentin (15 mg/kg daily) or placebo was given by a nasogastric
tube for 7 days before switching to the alternate treatment.44 There was prompt
substantial and significant relief of pain and reduction in the need for rescue
medication. In a similar study45 of 12 patients, greater pain relief was obtained
from carbamazepine (300 mg daily for 3 days) than from placebo. Excellent relief
of intractable and severe pain by epidural infusions of morphine (1- to 4-mg
morphine bolus injections every 8-24 hours) has been reported in a single case
study.46Opioid analgesics may aggravate autonomic gut dysmotility and bladder
distention.47,48

Recommendation
Simple analgesics or nonsteroidal anti-inflammatory drugs may be tried but often
do not provide adequate pain relief. Single small randomized controlled trials
support the use of gabapentin or carbamazepine in the intensive care unit for the
treatment of pain in the acute phase of GBS. Appropriate narcotic analgesics
may be used but require careful monitoring of adverse effects in the setting of
autonomic denervation. Adjuvant therapy with tricyclic antidepressant medication,
tramadol, gabapentin, carbamazepine, or mexilitene may aid in the long-term
management of neuropathic pain.

Management of bladder and bowel dysfunction


Constipation occurs frequently in bed-bound patients. Approximately half of the
patients develop adynamic ileus in the acute phase, often but not invariably in
conjunction with other features of dysautonomia.47 In other instances, the risk is

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increased by long-term immobilization, incremental doses of opiates for pain
control, or preexisting causes such as prior abdominal procedures.

Bladder function has only been studied infrequently in the acute phase of GBS,
partly because most patients are catheterized as part of their general nursing
care to maintain bodily hygiene and to avoid bladder distention. Voiding is more
frequently compromised with axonal types of GBS. Urodynamic studies have
documented bladder areflexia and disturbed bladder sensation.49

Recommendation
Daily abdominal auscultation for development of gut silence and monitoring of
opioid administration are recommended. In addition to suspension of gut-feeding
nasogastric and rectal tubes, erythromycin or neostigmine may be effective in
treating adynamic ileus.50 Promotility agents are contraindicated in patients with
dysautonomia. Bladder catheterization is often needed as part of the intensive
care of severely affected patients. A sterile, closed urinary drainage system
should be used with avoidance of breaking the seal to obtain urinary samples
and irrigation of the bladder.

Rehabilitation
Although most patients with GBS need rehabilitation, there are no long-term
rehabilitation outcome studies or comparisons of different methods.51 In
neuromuscular disease, overfatiguing the affected motor unit in therapy may
impede recovery and cause paradoxical weakening.52,53 Attention needs to be
paid to many details that cannot be summarized briefly. There is a danger of
muscle shortening and joint contractures.51 Prolonged immobilization leads to a
reduction of blood volume and increased episodes of postural hypotension.54 For
some immobilized patients, a tilt table has been useful.51 Weight loss and
significant sensory loss make patients susceptible to peripheral nerve
compression and the development of decubitus ulcers, requiring proper bed
positioning with frequent postural changes.51 In patients noted to have
immobilization hypercalcemia, early mobilization was correlated with a
therapeutic drop in the serum calcium levels.55 In the acute stage, patients lose
weight. During recovery, they regain weight owing to reduced activity levels.55,56

Recommendation
Treatment in the acute phase should include an individual program of gentle
strengthening involving isometric, isotonic, isokinetic, and manual resistive and
progressive resistive exercises. Rehabilitation should be focused on proper limb
positioning, posture, orthotics, and nutrition.

Management of fatigue

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A large proportion of patients with GBS remain seriously affected in their
psychosocial functioning even when their physical recovery was complete or
when they were left with only mild residual signs.57,58 Severe fatigue persists in
80% of patients and is unrelated to age, duration, or severity of the initial
illness.59 Frequency and severity of fatigue in GBS were comparable with that
encountered in other immune-mediated neuropathies.59,60 The cause and
contributing factors are not fully known, but fatigue appears in part to be a sequel
of forced inactivity and general muscle deconditioning. Both fatigue and
functional abilities were measurably improved with a supervised exercise
program in 2 single case studies.52,61 Pharmacological approaches are being
evaluated.62,63

Recommendation
An exercise program may be beneficial for persistent fatigue.

Future immunizations
Recurrence of GBS after immunization is rare.64 In response to a questionnaire,
11 (3.5%) of 311 patients reported recurrent symptoms within 6 weeks after
immunization, but it was possible to deduce with 95% confidence that the chance
of developing GBS severe enough to require hospital admission was less than
1.2%.65 In 2 previously reported cases, recurrence occurred following swine
influenza vaccine.66 Recurrent attacks of chronic inflammatory demyelinating
polyradiculoneuropathy have followed tetanus toxoid immunization.67,68

Recommendation
Immunizations are not recommended during the acute phase of GBS and
probably not during a period, possibly of 1 year, after the onset of the disease.
After that, immunizations need not be withheld, but the need for the immunization
should be reviewed on an individual basis. If GBS occurs within 6 weeks after a
particular immunization, consideration should be given to avoiding that
immunization in that individual in the future.

Conclusion
This review has highlighted the need for more research into all aspects of
supportive care for GBS.

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Article Information
Correspondence: Richard A. C. Hughes, MD, Department of Clinical
Neuroscience, Kings College, Guys Hospital, London SE1 1UL, England
(richard.a.hughes@kcl.ac.uk).

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Accepted for Publication: June 15, 2004.

Author Contributions:Study concept and design: Hughes, Wijdicks, Benson,


Cornblath, Hahn, Barohn, and Stevens. Acquisition of data: Hughes, Wijdicks,
Hahn, Meythaler, Sladky, and Stevens. Analysis and interpretation of data:
Hughes, Wijdicks, Cornblath, Hahn, Meythaler, and Stevens. Drafting of the
manuscript: Hughes, Wijdicks, Cornblath, Meythaler, and Sladky. Critical revision
of the manuscript for important intellectual content: Hughes, Wijdicks, Benson,
Cornblath, Hahn, Meythaler, Barohn, and Stevens. Statistical analysis:
Stevens. Administrative, technical, and material support: Hughes, Wijdicks,
Benson, and Stevens. Study supervision: Hughes and Wijdicks.

Disclaimer: Our recommendations must be interpreted in relation to the needs of


the individual patient and the capacity of the individual institution.

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