11/15/2020 Guillain-Barre Syndrome Treatment & Management: Approach Considerations, Prehospital and Emergency Department Care, ICU Trea…

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Guillain-Barre
Syndrome Treatment &
Management
Updated: Jun 24, 2020
Author: Michael T Andary, MD, MS; Chief Editor: Milton J Klein, DO, MBA more...

TREATMENT

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. [111]

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. [112]
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, [113] highlighting the need for further investigation of potential treatment
options.

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Prehospital and Emergency Department Care

Prehospital care of patients with Guillain-Barré 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. [114] 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. [115]
Declining NIF to -30 cm water should cause concern and very close monitoring. [116]

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. [88] 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

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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

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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. [117]

Mental status management

Hospitalized patients with GBS may experience mental status changes, including hallucinations,
delusions, vivid dreams, and sleep abnormalities. [118] 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. [119]

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
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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. [120]

In well-controlled clinical trials, the efficacy of IVIGs in GBS patients has been shown to equal that of
plasma exchange. [121, 122, 123, 124, 125, 126, 127, 128, 129]

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. [125, 130, 131]

Additionally, IVIG is the preferential treatment in hemodynamically unstable patients and in those
unable to ambulate independently. [132, 133] Some evidence suggests that in select patients who do
not respond initially to IVIG, a second dose may be beneficial. [134] 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. [132] 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. [132, 135] Immunotherapy

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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 [136, 137]
and to be safe in humans. [138, 139, 140] The Japanese Eculizumab Trial for GBS (JET-GBS), a
prospective, multicenter, placebo-controlled, double-blind, randomized phase 2 study, reported
eculizumab to be safe in severe cases of GBS. The investigators also found that at 24 weeks, 91.6%
of patients on eculizumab could walk independently and 74% could run, compared with 71.9% and
18%, respectively, for those on placebo. Nonetheless, the report noted that "[t]he primary outcome
measure did not reach the predefined response rate." Moreover, the study, which was designed to
evaluate safety, was considered underpowered with regard to showing efficacy. [139, 140]

Corticosteroids
Corticosteroids are ineffective as monotherapy. [1, 17, 141] According to moderate-quality evidence,
corticosteroids given alone do not significantly hasten recovery from GBS or affect the long-term
outcome. [141] According to low-quality evidence, oral corticosteroids delay recovery. [127, 141] 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. [141] In combination with IVIG, intravenous methylprednisolone may hasten recovery
but does not significantly affect long-term outcome. [127, 142]

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, 143] 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.

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Immune Adsorption
Immune adsorption is an alternative treatment for Guillain-Barré 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. [144]

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. [145]

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.

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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. [88, 89, 90] 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. [91, 92,
93] 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.

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