Guillain-Barré syndrome (GBS) is a heterogeneous grouping of immune-mediated processes generally characterized by motor, sensory, and autonomic dysfunction. In its classic form, GBS is an acute inflammatory demyelinating polyneuropathy characterized by progressive symmetric ascending muscle weakness, paralysis, and hyporeflexia with or without sensory or autonomic symptoms; however, variants involving the cranial nerves or pure motor involvement are not uncommon.
Emergency department care of patients with GBS involves close monitoring of respiratory and cardiovascular status. Intubation may be indicated.
Labile autonomic dysfunction may complicate the use of vasoactive and sedative drugs. In severe cases, muscle weakness may lead to respiratory failure. A 2008 epidemiological study reported 2-12% mortality despite ICU management.[1]
Plasma exchange and intravenous immune globulin may be effective, but patients may require prolonged intubation or intensive care. After discharge, outpatient physical therapy and occupational therapy may be beneficial in helping patients with GBS to regain their baseline functional status.[2, 3, 4]
Approximately half of all patients who have had GBS experience long-term residual neuropathy affecting large- and medium-sized myelinated fibers.[5] Overall, patients who have had GBS patients seem to have a reduced quality of life and physical functioning.[6] In extremely rare cases, patients may experience recurrent GBS.[7, 8]
Among survivors of GBS, Khan et al found that the following factors were associated with poorer level of functioning and well-being[9] :
Outcome does not appear to correlate with the severity of disease at onset.[9, 6]
See also Guillain-Barré Syndrome and Pediatric Guillain-Barré Syndrome.
For patient education information, see the Brain and Nervous System Center, as well as Guillain-Barré Syndrome.
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, IV, oxygen, and assisted ventilation may be indicated.[10] Intubation should be performed on patients who develop any degree of respiratory failure. Clinical indicators for intubation in the ED include hypoxia, rapidly declining respiratory function, poor or weak cough, and suspected aspiration. Typically, intubation is indicated when the forced vital capacity (FVC) is less than 15 mL/kg.[11]
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.
Only plasma exchange (PE) therapy and intravenous immune globulin (IVIG) have proven effective for Guillain-Barré syndrome (GBS). They may decrease autoantibody production and increase solubilization and removal of immune complexes. Both have been shown to shorten recovery time by as much as 50%. IVIG is easier to administer and has fewer complications than PE.[12, 13] The cost and efficacy of each are comparable.
Randomized trials in severe disease show that IVIG started within 4 weeks from onset hastens recovery as much as plasma exchange.[14, 12, 15, 16, 17] Combining PE and IVIG neither improved outcomes nor shortened illness duration.[18] IVIG has also been found safe and effective in the treatment of pediatric GBS.[18, 19]
Additionally, IVIG is the preferential treatment in hemodynamically unstable patients and in those unable to ambulate independently.[20, 18] Some evidence suggests that in select patients who do not respond initially to IVIG, a second dose may be beneficial.[21] However, this is not currently standard therapy and warrants further investigation.
Corticosteroids are ineffective as monotherapy.[22, 23, 24] According to moderate-quality evidence, corticosteroids given alone do not significantly hasten recovery from GBS or affect the long-term outcome.[24] According to low-quality evidence, oral corticosteroids delay recovery.[15, 24] Diabetes requiring insulin was significantly more common and hypertension less common with corticosteroids.
Substantial evidence shows that IV methylprednisolone alone produces neither significant benefit nor harm.[24] In combination with IVIG, IV methylprednisolone may hasten recovery but does not significantly affect long-term outcome.[15, 25]
In an animal model, intravenous eculizumab was reported to prevent respiratory paralysis and the functional and morphological hallmarks of terminal motor neuropathy. Human studies have not been reported.[26]
Interferon beta was not associated with significant clinical improvement compared with controls in a small randomized control trial.[27] Mycophenolate mofetil, used as an adjunct therapy with IVIG, showed no benefit in a pilot study.[28]
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.[29]
In critically ill patients, a small German study reported that treatment with selective immune adsorption (SIA) seems to be safe and effective.[30] In comparison to treatment with SIA only, sequential therapy with IVIG was not more effective.[30]
Simple analgesics or nonsteroidal anti-inflammatory drugs may be tried but often provide inadequate analgesia.
Single, small randomized controlled trials support the use of gabapentin or carbamazepine in the intensive care unit for management during the acute phase of GBS.
Adjuvant therapy with a tricyclic antidepressant, tramadol, gabapentin, carbamazepine, or mexiletine may aid in the long-term management of neuropathic pain.[3]
Venous thromboembolism is one of the major sequela of extremity paralysis. Time to development of deep vein thrombosis (DVT) or pulmonary embolism varies from 4-67 days following symptom onset.[3] Prophylaxis with gradient compression hose and subcutaneous low molecular weight heparin (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.
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] Any patients exhibiting clinical signs of respiratory compromise, in any degree, also should be admitted to an ICU.[3] In addition to those criteria used for intubation in the ED, Medical Research Council sum score as well as bulbar weakness have been reported to be predictive of requiring inpatient mechanical ventilation.[31]
The risk of sepsis and infection can be decreased by 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 may require prolonged intubation or prolonged intensive care.
The risk of deep venous thrombosis (DVT) and pulmonary embolism may be minimized by administration of heparin or a low molecular weight heparin and intermittent pneumatic compression devices.[3]
The use of cardiac telemetry and pacing in the case of severe bradycardia may help to reduce the risk of cardiac morbidity and mortality.[3]
Pain may be relieved by frequent passive limb movements, gentle massage, frequent position changes, and use of carbamazepine and gabapentin.[32, 3] Narcotics should be used judiciously because patients may already be at risk for ileus.[3]