Guillain-Barré syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy (affects nerve roots and peripheral nerves) with predominantly motor involvement. It is the most common cause of acute flaccid paralysis in the Western hemisphere and probably worldwide. By definition, maximal clinical weakness, the clinical nadir, occurs within 4 wk of disease onset. Several variations from the typical presentation of GBS exist (Box 1).

GBS

AIDP (acute inflammatory demyelinating polyradiculoneuropathy)

Acute polyneuropathy

Ascending paralysis

Postinfectious polyneuritis

• Symmetric weakness, most commonly involving proximal muscles initially, subsequently involving both proximal and distal muscles; difficulty in ambulating, getting up from a chair, or climbing stairs (Box 3)³
• Depressed or absent reflexes bilaterally
• Minimal to moderate glove and stocking paresthesias/dysesthesia/anesthesia or back pain¹
• Pain (caused by involvement of posterior nerve roots) may be prominent
• Autonomic abnormalities (bradyarrhythmias or tachyarrhythmias, hypotension or hypertension, constipation, urinary retention)⁴
• Respiratory insufficiency (caused by weakness of bulbar/intercostal muscles)
• Facial paresis, ophthalmoparesis, dysphagia (secondary to cranial nerve involvement)
• GBS consists of several clinical variants based on the pattern of clinical involvement and electrophysiologic findings. These include¹:
  1. AIDP (most common form in Europe and North America)
  2. Acute motor axonal neuropathy (AMAN; most prevalent form in China and Japan)
  3. Acute motor and sensory axonal neuropathy (AMSAN; has more severe sensory involvement and is associated with more severe clinical course and poorer prognosis)
  4. Miller Fisher syndrome (MFS; triad of ophthalmoplegia, ataxia, and areflexia)
  5. Acute pandysautonomia (rapid onset of parasympathetic and sympathetic failure without motor or sensory involvement)
  6. Regional variants (e.g., pharyngeal-cervical-brachial GBS, pure ataxic GBS)

• Unknown, but thought to be caused by infection-induced aberrant immune response.¹
• Preceding infectious illness 1 to 4 wk before disease onset has been noted. Antecedent events of GBS are summarized in Table 1.⁵ The most frequent antecedent infection is C. jejuni (associated with 30% of cases of GBS ).⁶
• Humoral and cell-mediated immune attack of peripheral nerve myelin, Schwann cells; sometimes with primary axonal involvement.¹

• Toxic peripheral neuropathies: Heavy metal poisoning (lead [microcytic anemia], thallium [alopecia], arsenic [typically accompanied by acute GI illness]), medications (vincristine, disulfiram), organophosphate poisoning, hexacarbon (glue sniffer neuropathy)
• Nontoxic peripheral neuropathies: Acute intermittent porphyria, fulminant vasculitic polyneuropathy, infectious (poliomyelitis, diphtheria, Lyme disease, West Nile virus), tick paralysis
• Neuromuscular junction disorders: Myasthenia gravis, botulism, snake envenomation
• Polymyositis and acute necrotizing myopathies
• Metabolic derangements such as hypermagnesemia, hypokalemia, hypophosphatemia
• Acute central nervous system (CNS) disorders such as basilar artery thrombosis with brain stem infarction, brain stem encephalomyelitis, transverse myelitis, or spinal cord compression
• Conversion disorder
• Malingering
• Table 2 differentiates clinical patterns of acute neuromuscular weakness

• Exclude other causes based on clinical history, examination, and laboratory tests.
• Lumbar puncture (may be normal in the first 1 to 2 wk of the illness). Typical findings include elevated cerebrospinal fluid (CSF) protein with few mononuclear leukocytes (albuminocytologic dissociation) in 80% to 90% of patients. Elevated CSF cell counts is an expected characteristic in cases associated with HIV seroconversion.
• Electromyography (EMG)/nerve conduction study (NCS) may be normal in the first 10 to 14 days of the disease. The earliest electrodiagnostic abnormality is prolongation or absence of H-reflexes. NCS evidence of demyelination (prolonged distal latency, conduction velocity slowing, conduction block, temporal dispersion, and prolonged F-waves) in two or more motor nerves confirms diagnosis of AIDP in the appropriate clinical context.

• CBC may reveal early leukocytosis with left shift. Electrolytes are tested to exclude metabolic causes of weakness.
• Heavy metal testing; urine porphyria screen; creatine kinase; HIV testing, including tests for HIV seroconversion, especially if CSF demonstrates lymphocytic pleocytosis. MRI of brain and spinal cord with and without contrast may be indicated if diagnosis is uncertain. In GBS, a gadolinium-enhanced MRI of the lumbosacral spine may reveal nerve root enhancement.
• Antibodies against ganglioside GQ1b may be present in up to 90% of patients with MFS. Immunoglobulin G antibodies against ganglioside GM1 may be associated with AMAN. There are no antiganglioside antibodies commonly associated with AIDP.
• In equivocal cases, especially if peripheral nerve vasculitis is a concern, nerve biopsy may aid in confirming a diagnosis of GBS. Sensory nerve biopsy demonstrates segmental demyelination with infiltration of monocytes and T cells into the endoneurium. Axonal loss is commonly seen in sensory nerve biopsy specimens in GBS.

• Close monitoring of respiratory function (frequent measurements of vital capacity, negative inspiratory force, and tidal volume) and pulmonary toilet should be done because respiratory failure is the major complication in GBS
• Frequent repositioning of patient to minimize formation of pressure sores
• Prevention of venous thromboembolism with antithrombotic stockings as a supplement to pharmacologic venous thromboembolism prevention
• Emotional support and social counseling for patient and family
• Pain control
• Cardiac monitoring to detect arrhythmias
• Physical and occupational therapy once patient is medically stable and able to participate

• Option 1: Infusion of IV immunoglobulins (IVIG; 0.4 g/kg/day for 5 days). Always check serum IgA levels before infusion to prevent anaphylaxis in IgA-deficient patients.
• Option 2: Early therapeutic plasma exchange (TPE or plasmapheresis: 200 to 250 ml/kg over five sessions every other day), started within 7 days of onset of symptoms, is beneficial in reducing the need for mechanical ventilation in patients with rapidly progressive disease and results in improved rate of recovery. It is contraindicated in patients with cardiovascular disease (recent myocardial infarction [MI], unstable angina), active sepsis, and autonomic dysfunction.
• Both therapies are equally effective and may shorten recovery time by 50%. There is no proven benefit from combining IVIG and plasma exchange. Glucocorticoids are contraindicated.
• Mechanical ventilation may be needed if force vital capacity (FVC) is <12 to 15 ml/kg, vital capacity is rapidly decreasing or is <1000 ml, negative inspiratory force is <20 cm H₂O, PaO₂ is <70, or the patient is having significant difficulty clearing secretions or is aspirating.

• Ventilatory support may be necessary in 10% to 20% of patients. Adequate fluid/electrolyte support and nutrition are necessary, especially in patients with dysautonomia or bulbar dysfunction.
• Aggressive nursing care to prevent decubitus, infections, fecal impactions, and pressure nerve palsies.
• Monitoring and treatment of autonomic dysfunction (bradyarrhythmias or tachyarrhythmias, orthostatic hypotension, systemic hypertension).
• Treatment of back pain and dysesthesia with neuropathic pain medications such as tricyclic antidepressants or gabapentin. Opiate narcotics can be used cautiously in the short term but may compound dysautonomia.
• Pharmacologic venous thromboembolism prevention with agents such as heparin (5000 U SC q12h) or enoxaparin (40 mg /day SC) in poorly ambulating and nonambulatory patients.
• Stress ulcer prevention in patients receiving ventilator support.
• Physical and occupational therapy rehabilitation, including supportive devices.

• Mortality rate is approximately 3% to 7% worldwide.¹ Causes of death include cardiac arrest, pulmonary embolism, and fulminant infections.
• Complete recovery by 200 days is seen in 80% of patients.⁴
• 65% with incomplete recovery and mild residual symptoms.⁴
• Predictors for poor recovery (inability to walk independently at 1 yr): Age >60 yr, preceding diarrheal illness, recent CMV infection, fulminant or rapidly progressing course, ventilatory dependence, reduced motor amplitudes (<20% normal), or unexcitable nerves on NCS. Outcomes also may be influenced by complications of medical therapy.⁴
• GBS is a monophasic illness.¹

• Neurology to aid in diagnosis and direct treatment
• Pulmonary/critical care for intensive care unit (ICU) management
• Otolaryngology or general surgery for tracheostomy in patients requiring prolonged ventilatory support
• Gastroenterology for percutaneous endoscopic gastrostomy for patients with prolonged inability to obtain nutrition orally

Guillain-Barré Syndrome (Patient Information)