Guillain-Barre Syndrome Treatment &

Management
Approach Considerations

Patients who are diagnosed with GBS should be admitted to a hospital for close
monitoring until it has been determined that the course of the disease has reached
a plateau or undergone reversal. Although the weakness may initially be mild and
nondisabling, symptoms can progress rapidly over just a few days. Continued
progression may result in a neuromuscular emergency with profound paralysis,
respiratory insufficiency, and/or autonomic dysfunction with cardiovascular
complications.

Approximately one third of patients require admission to an ICU, primarily because

of respiratory failure. After medical stabilization, patients can be treated on a
general medical/neurologic floor, but continued vigilance remains important in
preventing respiratory, cardiovascular, and other medical complications. Patients
with persistent functional impairments may need to be transferred to an inpatient
rehabilitation unit.

Continued care also is needed to minimize problems related to immobility,

neurogenic bowel and bladder, and pain. Early involvement of allied health staff is
recommended.

Early recognition and treatment of GBS also may be important in the long-term
prognosis, especially in the patient with poor clinical prognostic signs, such as older
age, a rapidly progressing course, and antecedent diarrhea. [103]

Immunomodulatory treatment has been used to hasten recovery. Intravenous

immunoglobulin (IVIG) and plasma exchange have proved equally effective.

Corticosteroids (oral and intravenous) have not been found to have a clinical benefit
in GBS. [104] Consequently, this class of drugs is not currently employed in
treatment of the syndrome.

A few studies have investigated other medications to treat GBS; however, the trials
have been small and the evidence weak, [105] highlighting the need for further
investigation of potential treatment options.

Prehospital and Emergency Department Care

Prehospital care of patients with Guillain-Barr syndrome (GBS) requires careful

attention to airway, breathing, and circulation (ABCs). Administration of oxygen and
assisted ventilation may be indicated, along with establishment of intravenous
access. Emergency medical services personnel should monitor for cardiac
arrhythmias and transport expeditiously.

In the emergency department (ED), continuation of ABCs, intravenous treatment,

oxygen, and assisted ventilation may be indicated. [106] Intubation should be
performed on patients who develop any degree of respiratory failure. Clinical
indicators for intubation in the ED include the following:

Hypoxia

Rapidly declining respiratory function

Poor or weak cough

Suspected aspiration

Typically, intubation is indicated when the forced vital capacity (FVC) is less than 15
mL/kg. [107] Declining NIF to -30 cm water should cause concern and very close
monitoring. [108]

Patients should be monitored closely for changes in blood pressure, heart rate, and
arrhythmias. Treatment is rarely needed for tachycardia. Atropine is recommended
for symptomatic bradycardia.

Because of the lability of dysautonomia, hypertension is best treated with short-

acting agents, such as a short-acting beta blocker or nitroprusside. Hypotension
from dysautonomia usually responds to intravenous fluids and supine positioning.
Temporary pacing may be required for patients with second- and third-degree heart
block.

Consult a neurologist if any uncertainty exists as to the diagnosis. Consult the ICU
team for evaluation of need for admission to the unit.

ICU Treatment

Good supportive care is critical in the treatment of patients with GBS. [81]
Admission to the ICU should be considered for all patients with labile dysautonomia,
a forced vital capacity of less than 20 mL/kg, or severe bulbar palsy. [3, 4] Any
patients exhibiting clinical signs of respiratory compromise to any degree also
should be admitted to an ICU. [3]

Because most deaths related to GBS are associated with complications of

ventilatory failure and autonomic dysfunction, many patients need to be monitored
closely in ICUs by physicians experienced in acute neuromuscular paralysis and its
accompanying complications.

Competent intensive care includes the following features:

Respiratory therapy

Cardiac monitoring

Safe nutritional supplementation

Monitoring for infectious complications (eg, pneumonia, urinary tract infections,

septicemia)

Respiratory therapy

Approximately one third of patients with GBS require ventilatory support. Monitoring
for respiratory failure, bulbar weakness, and difficulties with swallowing help to
anticipate complications. Proper positioning of the patient to optimize lung
expansion and secretion management for airway clearance is required to minimize
respiratory complications.

Serial assessment of ventilatory status is needed, including measurements of vital

capacity and pulse oximetric monitoring. Respiratory assistance should be
considered when the expiratory vital capacity decreases to less than 18 mL/kg or
when a decrease in oxygen saturation is noted (arterial PO2 < 70 mm Hg).
Tracheotomy may be required in a patient with prolonged respiratory failure,
especially if mechanical ventilation is required for more than 2 weeks.

Cardiac monitoring

Close monitoring of heart rate, blood pressure, and cardiac arrhythmias allows early
detection of life-threatening situations. Critically ill patients require continuous
telemetry and close medical supervision in an ICU setting. [3] Antihypertensives and
vasoactive drugs should be used with caution in patients with autonomic instability.
Hemodynamic changes related to autonomic dysfunction are usually transitory, and
patients rarely require long-term medications to treat blood pressure or cardiac
problems.

Nutrition

Enteral or parenteral feedings are required for patients on mechanical ventilation to

ensure that adequate caloric needs are met when the metabolic demand is high.
Even patients who are off the ventilator may require nutritional support if dysphagia
is severe. Precautions against dysphagia and dietary manipulations should be used
to prevent aspiration and subsequent pneumonias in patients at risk.

Prevention of infection

The risk of sepsis and infection can be decreased by the use of minimal sedation,
frequent physiotherapy, and mechanical ventilation with positive end-expiratory
pressure where appropriate. [3] Transfer may be appropriate if a facility does not
have the proper resources to care for patients who require prolonged intubation or
prolonged intensive care.

Prevention of thromboses, pressure sores, and contractures

Prevention of secondary complications of immobility is also required. Subcutaneous

unfractionated or low molecular-weight heparin (LMWH) and thromboguards are
often used in the treatment of immobile patients to prevent lower-extremity deep
venous thrombosis (DVT) and consequent pulmonary embolism (PE).

Prevention of pressure sores and contractures entails careful positioning, frequent

postural changes, and daily range-of-motion (ROM) exercises.

Bowel and bladder management

Although bowel and bladder dysfunction is generally transitory, management of

these functions is needed to prevent other complications. Initial management
should be directed toward safe evacuation and the prevention of overdistention.
Monitoring for secondary infections, such as urinary tract infection, also is an area
of concern. Nephropathy has been reported in pediatric patients. [109]

Mental status management

Hospitalized patients with GBS may experience mental status changes, including
hallucinations, delusions, vivid dreams, and sleep abnormalities. [110] These
occurrences are thought to be associated with autonomic dysfunction and are more
frequent in patients with severe symptoms. Such problems resolve as the patient
recovers. Psychiatric and psychological problems such as depression and anxiety
are likely to occur. Education, counseling, and medications are necessary to manage
these problems and help the patient adjust and improve from their profound
disability.
Physical Therapy

Estimates suggest that approximately 40% of patients who are hospitalized with
GBS require inpatient rehabilitation. Unfortunately, no long-term rehabilitation
outcome studies have been conducted, and treatment is often based on
experiences with other neurologic conditions. The goals of the therapy programs are
to reduce functional deficits and to target impairments and disabilities resulting
from GBS.

Early in the acute phase of GBS, patients may not be able to fully participate in an
active therapy program. At that stage, patients benefit from daily ROM exercises
and proper positioning to prevent muscle shortening and joint contractures.
Addressing upright tolerance and endurance also may be a significant issue during
the early part of rehabilitation.

Active muscle strengthening can then be slowly introduced and may include
isometric, isotonic, isokinetic, or progressive resistive exercises. Mobility skills, such
as bed mobility, transfers, and ambulation, are targeted functions. Patients should
be monitored for hemodynamic instability and cardiac arrhythmias, especially upon
initiation of the rehabilitation program. The intensity of the exercise program also
should be monitored, because overworking the muscles may, paradoxically, lead to
increased weakness.

In a study by Gupta et al in 35 patients (27 with classic GBS and 8 with acute motor
axonal neuropathy [AMAN]), GBS-related deficits included neuropathic pain
requiring medication therapy (28 patients), foot drop necessitating ankle-foot
orthosis (AFO) use (21 patients), and locomotion difficulties requiring assistive
devices (30 patients). At 1-year follow-up, the authors found continued foot drop in
12 of the AFO patients. However, significant overall functional recovery had
occurred within the general cohort. [111]

Occupational and Recreational Therapy

Occupational therapy professionals should be involved early in the rehabilitation

program to promote upper body strengthening, ROM, and activities that aid
functional self care. Restorative and compensatory strategies can be used to
promote functional improvements. Energy conservation techniques and work
simplification also may be helpful, especially if the patient demonstrates poor
strength and endurance.

Participation in recreational therapy assists in the patient's adjustment to disability

and improves integration into the community. Recreational activities, either new or
adapted, can be used to promote the growth, development, and independence of a
long-term hospital patient.

Speech Therapy
Speech therapy is aimed at promoting speech and safe swallowing skills for patients
who have significant oropharyngeal weakness with resultant dysphagia and
dysarthria. In ventilator-dependent patients, alternative communication strategies
also may need to be implemented.

Once weaned from the ventilator, patients with tracheostomies can learn voicing
strategies and can eventually be weaned from the tracheostomy tube. Cognitive
screening also can be performed conjointly with neuropsychology to assess for
deficits, since cognitive problems have been reported in some patients with GBS,
especially those who have had an extended ICU stay.

Immunotherapy

Plasma exchange carried out over a 10-day period may aid in removing
autoantibodies, immune complexes, and cytotoxic constituents from serum and has
been shown to decrease recovery time by 50%. A review of 6 randomized,
controlled trials involving 649 participants found that plasma exchange helped
speed recovery from GBS without causing harm, apart from being followed by a
slightly increased risk of relapse. [112]

In well-controlled clinical trials, the efficacy of IVIGs in GBS patients has been shown
to equal that of plasma exchange. [113, 114, 115, 116, 117, 118, 119, 120, 121]

IVIG treatment is easier to implement and potentially safer than plasma exchange,
and the use of IVIGs versus plasma exchange may be a choice of availability and
convenience. [117, 122, 123]

Additionally, IVIG is the preferential treatment in hemodynamically unstable

patients and in those unable to ambulate independently. [124, 125] Some evidence
suggests that in select patients who do not respond initially to IVIG, a second dose
may be beneficial. [126] However, this is not currently standard therapy and
warrants further investigation.

Combining plasma and IVIG has not been found to improve outcomes or shorten
illness duration in GBS. [124] However, some clinicians prefer to try plasma
exchange first, and if this does not provide patient improvement then they go to
IVIG. Theoretically, if IVIG is given first, then the plasma exchange will be removing
the IVIG, which was just given days earlier. There are no randomized controlled
trials that allow one to decide on the best plan.

Immunotherapy for children with GBS has not been rigorously studied with
randomized, well-controlled studies, but it is a standard aspect of treatment in this
age group. [124, 127] Immunotherapy for pregnant women has not been studied,
and safety for use during pregnancy has not been established.

Other possible treatments modulating the immune system include complement

inhibitors such as eculizumab. This has been shown to be effective in animal models
of Miller-Fisher syndrome [128, 129] and to be safe in humans [130] but have not
been subjected to a controlled trial.

Corticosteroids

Corticosteroids are ineffective as monotherapy. [1, 17, 131] According to moderate-

quality evidence, corticosteroids given alone do not significantly hasten recovery
from GBS or affect the long-term outcome. [131] According to low-quality evidence,
oral corticosteroids delay recovery. [119, 131] Diabetes requiring insulin was
significantly more common and hypertension less common with corticosteroids.

Methylprednisolone

Substantial evidence shows that intravenous methylprednisolone alone produces

neither significant benefit nor harm. [131] In combination with IVIG, intravenous
methylprednisolone may hasten recovery but does not significantly affect long-term
outcome. [119, 132]

Analgesia

Pain medications may be required in inpatient and outpatient settings. A tiered

pharmacologic approach that starts with nonsteroidal anti-inflammatory drugs
(NSAIDs) or acetaminophen, with narcotic agents added as needed, is usually
recommended.

Narcotics should be used judiciously because patients may already be at risk for
ileus. [3] Most patients do not require narcotic analgesics after the first couple of
months of illness.

Adjunct medications for pain, such as tricyclic antidepressants and certain

anticonvulsants, may be beneficial for dysesthetic-type pains. [3, 133] Single small,
randomized, controlled trials support the use of gabapentin or carbamazepine in the
ICU for management during the acute phase of GBS.

Nonpharmacologic pain relief therapies include frequent passive limb movements,

gentle massage, and frequent position changes. Desensitization techniques can be
used to improve the patient's tolerance for activities. Modalities such as
transcutaneous electrical nerve stimulation (TENS) and heat may prove beneficial in
the management of myalgia. Education and psychological counseling can decrease
the amount of suffering associated with this pain and disability.

Immune Adsorption

Immune adsorption is an alternative treatment for Guillain-Barr syndrome that is

still in the early stages of investigation. A small, prospective study reported no
difference in outcome between patients treated with immunoadsorption and those
treated with plasma exchange. [134]

In critically ill patients, a small German study reported that treatment with selective
immune adsorption (SIA) seemed to be safe and effective. In comparison with
treatment with SIA only, sequential therapy with IVIG was not more effective. [135]

Prevention of Thromboembolism

Venous thromboembolism is one of the major sequelae of extremity paralysis. Time

to development of DVT or pulmonary embolism varies from 4-67 days following
symptom onset. [3] Prophylaxis with gradient compression hose and subcutaneous
LMWH may dramatically reduce the incidence of venous thromboembolism. [3]

True gradient compression stockings (30-40 mm Hg or higher) are highly elastic and
provide compression along a gradient that is highest at the toes and gradually
decreases to the level of the thigh. This reduces capacity venous volume by
approximately 70% and increases the measured velocity of blood flow in the deep
veins by a factor of 5 or more.

The ubiquitous white stockings known as antiembolic stockings or thromboembolic

disease (TED) hose produce a maximum compression of 18 mm Hg and rarely are
fitted in such a way as to provide adequate gradient compression. They have not
been shown to be effective as prophylaxis against thromboembolism.

Consultations

Consultation with a neurologist can be helpful in the initial diagnosis, workup, and
treatment of patients admitted to the medical floor with GBS.

Critical care specialists may be required for patients in the ICU to help manage
respiratory failure and multiple medical complications.

Consultation with a pulmonologist may be needed to perform workup and to

manage respiratory issues, such as acute respiratory distress syndrome (ARDS),
pneumonia, and respiratory failure.

Consultation with a cardiologist may be required if significant cardiovascular

complications, such as labile blood pressure and cardiac arrhythmias, arise from the
associated autonomic dysfunction.

Consultation with a surgeon may be required for the placement of tracheostomies,

enteral feeding tubes, and central lines.

Physical medicine and rehabilitation specialists (physiatrists) should evaluate

patients for impairments and disabilities arising from GBS and should help to
determine the most appropriate setting for and intensity of rehabilitation care and
assist with their rehabilitation and return to function.
Long-Term Monitoring

Although follow-up studies generally have assessed patients 6-12 months after
onset of GBS, some studies have reported continued improvements in strength even
beyond 2 years. With prolonged recovery possible, GBS patients with continued
neurologic deficits may benefit from ongoing physical therapy and conditioning
programs.

As previously mentioned, numerous papers have addressed the issue of persistent

fatigue after recovery from GBS. [81, 82, 83] Studies have suggested that a large
percentage of patients continue to have fatigue-related problems, subsequently
limiting their function at home and at work, as well as during leisure activities.
Treatment suggestions range from gentle exercise to improvement in sleep patterns
to relief of pain or depression, if present.

GBS can produce long-lasting changes in the psychosocial status of patients and
their families. [84, 85, 86] Changes in work and leisure activities can be observed in
just over one third of these patients, and psychosocial functional health status can
be impaired even years after the GBS event.

Interestingly, psychosocial performance does not seem to correlate with the

severity of residual problems with physical function. Poor conditioning and easy
fatigability may be contributory factors. Therefore, providing long-term attention
and support for this population group is important.