Guillain-Barré Syndrome (Acute Idiopathic Polyneuropathy)

Essentials of Diagnosis

• Acute or subacute progressive polyradiculoneuropathy

• Weakness is more severe than sensory disturbances

• Acute dysautonomia may be life-threatening

General Considerations

• A symmetric sensory, motor, or mixed deficit, often most marked distally

• Probably has an immunologic basis, but the mechanism is unclear

• Sometimes follows infections, innoculations, or surgical procedures

• There is an association with preceding Campylobacter jejuni enteritis

• The axonal subtypes of the syndrome (acute motor axonal neuropathy [AMAN] and acute motor and sensory axonal neuropathy [AMSAN]) are caused by antibodies to gangliosides on the axon membrane, including anti-GM1, anti-GM1b, anti-GD1a, anti-GD1b, and (in AMAN) anti-GalNAC-GD1a antibodies

• The Miller Fisher syndrome is another subtype

  • Characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia

  • Associated with anti-GQ1b antibodies

Symptoms and Signs

Motor symptoms

• The main complaint is of weakness

  • Varies widely in severity in different patients

  • Often has a proximal emphasis and symmetric distribution

  • Usually begins in the legs, spreading to a variable extent but frequently involving the arms and often one or both sides of the face

  • The muscles of respiration or deglutition may also be affected

Sensory symptoms

• Sensory symptoms are usually less conspicuous than motor ones, but distal paresthesias and dysesthesias are common, and neuropathic or radicular pain is present in many patients

Autonomic symptoms

• Autonomic disturbances are common, may be severe, and are sometimes life-threatening; they include the following

  • Tachycardia

  • Cardiac rhythm irregularities

  • Hypotension or hypertension

  • Facial flushing

  • Abnormalities of sweating

  • Pulmonary dysfunction

  • Impaired sphincter control

Differential Diagnosis

• Chronic inflammatory demyelinating polyneuropathy (CIDP)

• Porphyria

• Diphtheritic neuropathy

• Toxic neuropathy, eg, lead, mercury, organophosphates, hexacarbon solvents

• HIV infection

• Poliomyelitis

• Botulism

• Tick paralysis

• Spinal cord lesion

• Transverse myelitis

• West Nile virus infection

• Periodic paralysis syndrome

Laboratory Tests

• The cerebrospinal fluid characteristically contains a high protein concentration with a normal cell count, but this change may take up to 2 weeks to develop

• White blood cell counts > 50 cells/mcL should prompt consideration of alternative diagnoses

Diagnostic Procedures

• Electrophysiologic (nerve conduction) studies may reveal marked abnormalities, which do not necessarily parallel the clinical disorder in their temporal course

Medications

• Marked hypotension may respond to volume replacement or pressor agents

• Intravenous immunoglobulin (400 mg/kg/day for 5 days) is helpful

• Low-dose heparin to prevent pulmonary embolism should be considered

• Prednisone is ineffective and may prolong recovery time

Therapeutic Procedures

• Respiratory toilet and chest physical therapy help prevent atelectasis

• Plasmapheresis is of value

  • Best performed within the first few days of illness

  • Best reserved for clinically severe or rapidly progressive cases or those with ventilatory impairment

Follow-Up

• Monitor spirometry

  • Patients should be admitted to intensive care units if their forced vital capacity is declining

  • Intubation is considered if the forced vital capacity reaches 15 mL/kg, the mean inspiratory force reaches –30 mm Hg dyspnea becomes evident, or oxygen saturation declines

Prognosis

• Most patients eventually make a good recovery

  • However, recovery may take many months

  • About 20% of patients have residual disability

• Approximately 3% of patients have one or more clinically similar relapses, sometimes several years after the initial illness

When to Refer

• All patients should be referred

When to Admit

• All patients should be hospitalized until their condition is stable and there is no respiratory compromise