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. [115]
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. [116] 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, [117] highlighting the need for further investigation of potential treatment options.
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. [118] Intubation should be performed on patients who develop any degree of respiratory failure. Clinical indicators for intubation in the ED include the following:
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Hypoxia
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Rapidly declining respiratory function
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Poor or weak cough
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Suspected aspiration
Typically, intubation is indicated when the forced vital capacity (FVC) is less than 15 mL/kg. [119] Declining NIF to -30 cm water should cause concern and very close monitoring. [120]
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. [92] 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:
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Respiratory therapy
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Cardiac monitoring
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Safe nutritional supplementation
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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. [121]
Mental status management
Hospitalized patients with GBS may experience mental status changes, including hallucinations, delusions, vivid dreams, and sleep abnormalities. [122] 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. [123]
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. [124]
In well-controlled clinical trials, the efficacy of IVIGs in GBS patients has been shown to equal that of plasma exchange. [125, 126, 127, 128, 129, 130, 131, 132, 133]
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. [129, 134, 135]
Additionally, IVIG is the preferential treatment in hemodynamically unstable patients and in those unable to ambulate independently. [136, 137] Some evidence suggests that in select patients who do not respond initially to IVIG, a second dose may be beneficial. [138] 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. [136] 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. [136, 139] 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 [140, 141] and to be safe in humans. [142, 143, 144] 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. [143, 144]
Corticosteroids
Corticosteroids are ineffective as monotherapy. [1, 17, 145] According to moderate-quality evidence, corticosteroids given alone do not significantly hasten recovery from GBS or affect the long-term outcome. [145] According to low-quality evidence, oral corticosteroids delay recovery. [131, 145] 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. [145] In combination with IVIG, intravenous methylprednisolone may hasten recovery but does not significantly affect long-term outcome. [131, 146]
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, 147] 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-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. [148]
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. [149]
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. [92, 93, 94] 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. [95, 96, 97] 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.
