Guillain-Barré syndrome

• Landry-Guillain-Barré-Strohl syndrome
• Acute inflammatory demyelinating polyneuropathy
• Acute demyelinating polyneuritis

• 357.0 Acute infective polyneuritis

• G61.0 Guillain-Barré syndrome

• 5G: Impaired motor function and sensory integrity associated with acute or chronic polyneuropathies1

Description

• Demyelination of the peripheral nervous system, resulting in quickly progressing paralysis beginning distally, potentially ending full quadriplegic presentation with respiratory failure2,3,4

Essentials of Diagnosis

• Most common bacteria identified with Guillain-Barré syndrome (GBS) is Campylobacter jejuni; other common infections include cytomegalovirus and Epstein-Barr virus
• 60% of cases have preceding upper-respiratory illness or vaccination 1 to 3 weeks prior to onset

General Considerations

• Two forms of GBS are common, though several other variants exist
  • Non-axonal form
    • Peripheral nerves undergo demyelination, but no damage to axons
    • High likelihood of regaining full function with appropriate medical and therapeutic intervention
  • Axonal form
    • Axon of the peripheral nerve is damaged, does not re-myelinate
    • Function through that nerve is lost, patient will likely have permanent functional loss of associated muscle
• Rapid progression from onset to potential respiratory failure; should be identified and treated very quickly
• Most patients show gradual return of strength beginning 2 to 4 weeks after the point at which progression of the disease stops (“nadir”)
• Approximately 50% of patients experience dysfunction of the autonomic nervous system, including
  • Low cardiac output
  • Cardiac dysrhythmias
  • Marked fluctuation in blood pressure
  • Poor venous return
  • Bowel and bladder retention

Demographics

• Males and females of all ages susceptible
• Approximately 4 in 100,000 people afflicted each year

Signs and Symptoms

• Progressive weakness in distal to proximal direction resulting in quadriplegia
  • Decreased muscle strength, recruitment, endurance
  • Decreased active range of motion (AROM)
  • Decreased sensation in stocking/glove pattern
• Loss of deep tendon reflexes, initially at the calcaneal (Achilles) tendon
• Deep muscle aching
• Respiratory failure

Functional Implications

• Bed mobility, transfers, gait, basic and complex ADLs
• Decreased independence with all self-care
• Decreased ability to interact with environment physically, verbally, sometimes visually (if cranial nerves controlling vision are affected)

Possible Contributing Causes

• Definitive cause unknown
• Campylobacter jejuni most common bacterium associated with GBS
• Pre-morbid respiratory illness (severe cold or flu) is also a common contributing cause

Differential Diagnosis

• Spinal cord compression (myelopathy)
• Miller-Fisher syndrome (GBS variant)
• Chronic inflammatory demyelinating polyradiculoneuropathy
• Acute spinal cord disease
• Myasthenia gravis
• Sarcoidosis
• Upper motor neuron (UMN) disorder
• Tick paralysis
• Poliomyelitis
• Polyarteritis nodosa
• Polyneuropathy of critical illness
• Transverse myelitis
• Cervical stenosis
• Spinal tumor
• Diabetic neuropathy

Laboratory Tests

• Test of cerebral spinal fluid (CSF): positive if protein levels elevated

Imaging

• MRI with contrast ...