Guillain-Barré Syndrome

Published on 07/03/2015 by admin

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Chapter 63 Guillain-Barré Syndrome

Ala Nozari, MD, PhD , Corey R. Fehnel, MD , Lee H. Schwamm, MD, FAHA

1 What is Guillain-Barré syndrome (GBS)?

Also known as acute polyradiculoneuritis, GBS is an acute onset of peripheral neuropathy with progressive muscle weakness and areflexia that reaches maximum over 3 days to 3 weeks. It has a number of variants, including pure motor or motor-sensory variants, Miller Fisher variant, bulbar variant, and primary axonal GBS.

2 Describe the pathophysiology of GBS

GBS is a collection of syndromes with inflammatory demyelinating polyradiculoneuropathy. It is triggered by both humoral and cell-mediated autoimmune response to an immune sensitizing event such as an upper respiratory tract infection or cytomegalovirus, herpes simplex virus, Campylobacter jejuni, or mycoplasma infections. A clear antecedent infection is often difficult to identify. Antibodies to gangliosides and glycolipids trigger myelin disruption in the peripheral nervous system. Axonal antibodies occur in some cases, typically after campylobacter infection, and carry a worse prognosis for complete recovery. An increased incidence has been reported in patients with lymphoma or lupus. In the United States, it is a sporadic disease, but variants in Asia are often epidemic in the summer months, presumably because of increased human exposure to zoonotic infections from domesticated livestock.

3 What is the typical clinical presentation of GBS?

GBS is an ascending paralysis that is typically characterized by symmetric weakness, sensory dysesthesias, and hyporeflexia. Mild cases may present with only mild weakness or as variants (e.g., ataxia, ophthalmoplegia, and hyporeflexia) without significant appendicular weakness. Fulminant cases may cause severe ascending weakness leading to complete tetraplegia and with paralysis of cranial nerves and respiratory muscles (involvement of the phrenic and intercostal nerves).

4 Describe the diagnostic criteria for GBS

Clinical features required for the diagnosis of GBS include areflexia and progressive motor weakness of more than one limb. Features strongly supportive of the diagnosis include progression of the motor weakness, relative symmetry, cranial nerve involvement, mild sensory symptoms, autonomic dysfunction, and recovery within 2 to 4 weeks after progression stops.

5 What is the differential diagnosis of subacutely evolving, generalized motor weakness?

Disorders frequently mimicking GBS include acute intermittent porphyria, transverse myelitis, tick paralysis, myasthenia gravis, hypophosphatemia, and carcinomatous or lymphomatous meningitis. Severe peripheral neuropathies due to vasculitis or toxins such as lead, nitrofurantoin, dapsone, thallium, arsenic, and many others should also be considered.

6 Describe laboratory and radiologic findings for GBS

Cerebrospinal fluid (CSF) analysis shows elevated protein level without pleocytosis (albuminocytologic dissociation). Electromyogram and nerve conduction study results may be normal in the early acute period but after 1 to 2 weeks reveal characteristic segmental demyelination and reduction of conduction velocity. Dispersion or absence of F waves is an important early finding that is indicative of root demyelination. Although magnetic resonance imaging provides no characteristic findings, diffuse enhancement of cauda equina and nerve roots may occur as a result of disruption of the blood-nerve barrier caused by the inflammation. Conspicuous nerve root enhancement correlates with pain, disability grade, and duration of recovery.

7 Describe the initial management of GBS in patients

Patients with GBS are often admitted to an intensive care unit for close observation, as some may have respiratory failure, autonomic instability, or hypotension during plasmapheresis. Patients with early cranial nerve dysfunction are more susceptible to aspiration and dysautonomia. Early tracheostomy should be considered in patients with severe weakness, particularly if it involves the bulbar musculature. Radicular thoracolumbar pain is often prominent and may benefit from treatment with neuropathic pain agents such as gabapentin. Aggressive skin care, physical therapy, and splinting are important to prevent skin breakdown and contractures. Low-molecular-weight heparin and graduated pressure stockings should be used to prevent deep vein thrombosis.

8 When should patients with GBS have an endotracheal tube placed?

Respiratory failure is often caused by a combination of respiratory muscle insufficiency and difficulty clearing secretions or inability to cough and protect the airway. This can be complicated by aspiration and respiratory tract infections. Bedside pulmonary function testing is helpful but may be difficult in patients with significant facial weakness because of the decreased ability to form a good seal. Clinical symptoms of neuromuscular respiratory failure include rapid and shallow breathing, restlessness, sweating, and increased accessory muscle use with the presence of paradoxical abdominal movements during inspiration.

Indications for intubation are not always clear, but the decision is supported by the presence of severe bulbar weakness with difficulty to handle secretions and protect airway, and rapidly evolving motor weakness. A vital capacity of less than 15 to 20 mL/kg and a maximum negative inspiratory pressure of less than −20 mm Hg can also indicate need for intubation. Because these patients often have facial weakness that prevents a tight seal around the lips, surrogate measures of vital capacity such as having the patient count out loud as high as he or she can in one breath may be useful (approximately 100 mL for each number counted slowly). Hypoxia by pulse oximetry or arterial blood gas measurements are only very late signs of respiratory muscle failure and if normal should not provide reassurance as to the stability of the condition of the patient with GBS. Hypercapnia and respiratory acidosis are also late signs of respiratory failure and should prompt rapid institution of ventilatory support.

9 What considerations regarding anesthesia and neuromuscular blockade should be kept in mind when performing endotracheal intubation in a patient with GBS?

Cranial and autonomic nervous dysfunction often predisposes these patients to an increased risk for aspiration. Aspiration precautions, including decompression of the stomach before the induction of anesthesia, should therefore be considered in all patients. A nondepolarizing neuromuscular blocking agent should be used whenever possible, as depolarizing neuromuscular blocking agents have been associated with an increased risk for hyperkalemia-induced cardiac arrest in immobilized or paralyzed patients.

10 Describe autonomic dysfunction and its clinical implications in GBS

Dysautonomia consists of rapid fluctuations in blood pressure, heart rhythm disturbances including sinus bradycardia, or even sinus arrest. Gastric motility can also be affected. Dysautonomia results from excessive or insufficient sympathetic or parasympathetic activity. It is more commonly seen with the demyelinating form, as opposed to the axonal form of GBS and is usually present if the disease is severe enough to require mechanical ventilation. The hemodynamic instability is typically short-lived and self-limited, but small doses of short-acting and titratable vasoactive medications may be required.

11 Do any specific therapies for GBS exist?

Supportive therapy, mechanical ventilation, and measures to prevent aspiration are important. An arterial catheter may be placed to monitor blood pressure if hemodynamic instability is anticipated. Though debate remains, immunoglobulins are considered first-line treatment, particularly in the setting of dysautonomia or if a significant risk exists for catheter-related complications with plasmapheresis or exchange transfusion. Intravenous immunoglobulin (IVIG) is typically administered at a dose of 0.4 g/kg per day for 5 days. Side effects include anaphylaxis, aseptic meningitis, acute renal failure, and thromboembolic events. Patients who have immunoglobulin A (IgA) deficiency should receive IVIG that is also IgA deficient to reduce the risk of adverse reaction. Early plasma exchange may augment the recovery and can reduce the residual deficit. Typically it consists of five exchanges of 50 mL/kg over a 90- to 120-minute period, with 5% albumin repletion. Side effects include hypovolemia and hemodynamic instability, vasovagal reactions, anaphylaxis, hemolysis, thrombocytopenia, bleeding, and hypocalcemia. Relative contraindications to treatment include sepsis, recent myocardial infarction, marked dysautonomia, and active bleeding. Steroids are not helpful.

12 Name other important components of the general care for patients with GBS

In case of facial dysplasia, the eyes should be protected from exposure keratitis. Aggressive skin care and measures to avoid pressure palsies of arms and legs are needed. Physical therapy and splinting are important to prevent contractures, and early enteral nutrition or total parenteral nutrition in patients with adynamic ileus should be considered. All patients require deep venous thrombosis prophylaxis with pneumatic compression devices and low-molecular-weight heparin, gastrointestinal prophylaxis, judicious use of analgesics and anxiolytics, and tremendous emotional support.

13 Describe the outcome and appropriate timing for transfer out of the intensive care unit

Patients are usually admitted to the intensive care unit because of hemodynamic instability and acute consequences of respiratory muscle weakness, including hypercapnic respiratory failure, pneumonia, atelectasis, and difficulty to protect the airway. After the peak phase of the illness has resolved the patient must be prepared for discharge with use of a multidisciplinary approach incorporating potential caregivers. Recovery may not be complete for several months, and residual weakness and atrophy are present in up to 35% of patients. Most patients, however, have excellent recovery in muscle function, eventually returning to their baseline state with or without areflexia.

Key Points Guillain-barré syndromeimage

1. GBS is an acute inflammatory demyelinating polyneuropathy with progressive muscle weakness and areflexia.

2. Cranial neuropathy is often present and may include facial diplegia, ophthalmoplegia, and bulbar symptoms.

3. Symptoms are often preceded by an infectious illness, immunization, or surgery.

4. Respiratory failure and autonomic dysfunction are major complications of GBS.

5. Diagnosis can be confirmed by clinical history, CSF findings showing albuminocytologic dissociation, and nerve conduction studies.

6. Specific therapies includes IVIG and plasmapheresis therapy.

Bibliography

1 Asahina M., Kuwabara S., Suzuki A., et al. Autonomic function in demyelinating and axonal subtypes of Guillain-Barré syndrome. Acta Neurol Scand. 2002;105:44–50.

2 Asbury A.K. Diagnostic considerations in Guillain-Barré syndrome. Ann Neurol 9 Suppl. 1981:1–5.

3 Gorson K.C., Ropper A.H., Muriello M.A., et al. Prospective evaluation of MRI lumbosacral nerve root enhancement in acute Guillain-Barré syndrome. Neurology. 1996;47:813–817.

4 Guillain-Barre Syndrome Steroid Trial Group. Double-blind trial of intravenous methylprednisolone in Guillain-Barré syndrome. Lancet. 1993;341:586–590.

5 Hughes R.A., Hadden R.D., Gregson N.A., et al. Pathogenesis of Guillain-Barré syndrome. J Neuroimmunol. 1999;100:74–97.

6 Hughes R.A., Raphaël J.C., Swan A.V., et al. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev. 1, 2006. CD002063, 2006

7 Hughes R.A., Wijdicks E.F., Benson E., et al. Supportive care for patients with Guillain-Barré syndrome. Arch Neurol. 2005;62:1194–1198.

8 Kehoe M. Guillain-Barré syndrome—a patient guide and nursing resource. Axone. 2001;22(4):16–24.

9 Lawn N.D., Fletcher D.D., Henderson R.D., et al. Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol. 2001;58:893–898.

10 Ropper A.H. Further regional variants of acute immune polyneuropathy. Bifacial weakness or sixth nerve paresis with paresthesias, lumbar polyradiculopathy, and ataxia with pharyngeal-cervical-brachial weakness. Arch Neurol. 1994;51:671–675.

11 Ropper A.H., Shahani B.T. Pain in Guillain-Barré syndrome. Arch Neurol. 1984;41:511–514.

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