Guillain-Barre syndrome

Background

• Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction. Although there is often a motor component, patients can also present with sensory deficits.
• 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 patients)
• Autonomic dysfunction occurs in 50% of patients

Variants and Subtypes

There are multiple Guillain Barre variants with differing presentaions

1. Acute inflammatory demyelinating polyneuropathy - most common type with progressive symmetric muscle weakness often with decreased deep tendon reflexes due to peripheral nerve involvment
2. Acute motor axonal neuropathy - often associated with campylobacter infections with only motor involvement
3. Acute motor and sensory axonal neuropathy - presence both motor and sensory involement
4. Miller-Fisher Syndrome - presence of ophthalmoplegia with ataxia and areflexia

Miller-Fisher Syndrome

• Associated with 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
• May present similarly to botulism, which is also descending paralysis

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)

Evaluation

Required

• Progressive weakness of more than one limb
• Areflexia

Suggestive

• Progression over days to weeks
• Recovery beginning 2–4 wk after cessation of progression
• Relative symmetry of symptoms
• Mild sensory signs and symptoms
• CN involvement (Bell's Palsy, dysphagia, dysarthria, ophthalmoplegia)
• Autonomic dysfunction
  • Tachycardia, bradycardia, dysrhythmias, wide variations in BP, postural hypotension
  • Urinary Retention
  • Constipation
  • Facial flushing
• Absence of fever at onset
• Albumin-cytological dissociation of CSF (high protein (>45) and low WBC count (<10)) is most common. However patients with HIV can have a pleocytosis
• Typical findings on electromyogram and nerve conduction studies
• MRI: Selective enhancement of the anterior spinal nerve roots is suggestive

Management

IVIG

• Treat nonambulatory patients within 2 weeks of symptom onset

IVIG vs Plasmapheresis

• IVIG associated with thromboembolism and aseptic meningitis
• Plasmapheresis associated with greater hemodynamic instability, lower rate of relapse
• Combined IVIG and plasmapheresis no better than single therapy (IVIG or plasmapheresis)
• IVIG preferred due to convenience and availability

Intubation indications

• Vital capacity <15mL/kg
• Negative Inspiratory Force < 30 cm H2O
• PaO2 <70 mm Hg on room air
• Bulbar dysfunction (difficulty with breathing, swallowing, or speech)
• Aspiration

• Avoid succinylcholine during intubation, as this may cause severe hyperkalemia

Disposition

Indications for admission to ICU

• Autonomic dysfunction
• Bulbar dysfunction
• Initial vital capacity <20 mL/kg
• Initial negative inspiratory force <–30 cm of water
• Decrease of >30% of vital capacity or negative inspiratory force
• Inability to ambulate
• Treatment with plasmapheresis
• Anticipated clinical course requiring mechanical ventilation

See Also

• Weakness