Guillain-Barre syndrome

Background

• Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction
• Associated with viral or febrile illness, campylobacter infection, or vaccination
• Symptoms at worst 2-4wk after onset, then plateau for 2-4wk, then remit from wks-months
• Associated with Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, and Mycoplasma pneumoniae

Clinical Features

• Viral illness -> ascending, symmetric weakness or paralysis and loss of DTRs
• Little or no sensory involvement
• May progress to diaphragm resulting in need for mechanical ventilation (33% of pts)
• Autonomic dysfunction occurs in 50% of pts

• Miller-Fisher Syndrome
  • Associated w/ campylobacter infection
  • More likely to be preceded by diarrhea than viral prodrome
  • Consists of ophthalmoplegia and ataxia
  • Weakness is less severe but DESCENDING; disease course milder than classic GBS

Diagnosis

1. Required
   1. Progressive weakness of more than one limb
   2. Areflexia
2. Suggestive
   1. Progression over days to weeks
   2. Recovery beginning 2–4 wk after cessation of progression
   3. Relative symmetry of symptoms
   4. Mild sensory signs and symptoms
   5. CN involvement (Bell's Palsy, dysphagia, dysarthria, ophthalmoplegia)
   6. Autonomic dysfunction
      1. Tachycardia, bradycardia, dysrhythmias, wide variations in BP, postural hypotension
      2. Urinary Retention
      3. Constipation
      4. Facial flushing
   7. Absence of fever at onset
   8. Albumin-cytological dissociation of CSF (high protein (>45) and low WBC count (<10))
   9. Typical findings on electromyogram and nerve conduction studies
   10. MRI: Selective enhancement of the anterior spinal nerve roots is suggestive

Differential Diagnosis

Weakness

• Neuromuscular weakness
  • Upper motor neuron:
    • CVA
    • Hemorrhagic stroke
    • Multiple sclerosis
    • Amyotrophic Lateral Sclerosis (ALS) (upper and lower motor neuron)
  • Lower motor neuron:
    • Spinal and bulbar muscular atrophy (Kennedy's syndrome)
  • Spinal cord disease:
    • Infection (Epidural abscess)
    • Infarction/ischemia
    • Trauma (Spinal Cord Syndromes)
    • Inflammation (Transverse Myelitis)
      • Acute flaccid myelitis
    • Degenerative (Spinal muscular atrophy)
    • Tumor
  • Peripheral nerve disease:
    • Guillain-Barre syndrome
    • Toxins (Ciguatera)
    • Tick paralysis
    • Diabetic peripheral neuropathy
  • Neuromuscular junction disease:
    • Myasthenia gravis crisis
    • Botulism
    • Organophosphate toxicity
    • Lambert-Eaton myasthenic syndrome
  • Muscle disease:
    • Rhabdomyolysis
    • Dermatomyositis
    • Polymyositis
    • Alcoholic myopathy
• Non-neuromuscular weakness
  • Can't miss diagnoses:
    • ACS
    • Arrhythmia/Syncope
    • Severe infection/Sepsis
    • Hypoglycemia
    • Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
      • Hypokalemic periodic paralysis
      • Thyrotoxic periodic paralysis
    • Respiratory failure
  • Emergent Diagnoses:
    • Symptomatic Anemia
    • Severe dehydration
    • Hypothyroidism
    • Polypharmacy
    • Malignancy
    • Aortic disease - occlusion, stenosis, dissection
  • Other causes of weakness and paralysis
    • Acute intermittent porphyria (ascending weakness)

Treatment

1. Intubation indications:
   1. Vital capacity <15mL/kg
   2. PaO2 <70 mm Hg on room air
   3. Bulbar dysfunction (difficulty with breathing, swallowing, or speech)
   4. Aspiration
2. IVIG OR plasmapheresis (provide equivalent but not additive effects)

Disposition

1. Indications for admission to ICU:
   1. Autonomic dysfunction
   2. Bulbar dysfunction
   3. Initial vital capacity <20 mL/kg
   4. Initial negative inspiratory force <–30 cm of water
   5. Decrease of >30% of vital capacity or negative inspiratory force
   6. Inability to ambulate
   7. Treatment with plasmapheresis
   8. Anticipated clinical course requiring mechanical ventilation

See Also

• Weakness

Source

• Tintinalli
• Bunney EB, Gallagher EJ: Peripheral Nerve Disorders, in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 7. St. Louis, Mosby, Inc., 2010, (Ch) 105:p 1400-1401.