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
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))Cite error: Invalid
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- MRI: Selective enhancement of the anterior spinal nerve roots is suggestiveCite error: Invalid
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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)
- Degenerative (Spinal muscular atrophy)
- Tumor
- Peripheral nerve disease:
- Neuromuscular junction disease:
- Muscle disease:
- Rhabdomyolysis
- Dermatomyositis
- Polymyositis
- Alcoholic myopathy
- Upper motor neuron:
- Non-neuromuscular weakness
- Can't miss diagnoses:
- ACS
- Arrhythmia/Syncope
- Severe infection/Sepsis
- Hypoglycemia
- Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
- 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)
- Can't miss diagnoses:
Treatment
- IVIG OR plasmapheresis (provide equivalent but not additive effects)
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
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