Guillain-Barre Syndrome - Summary
Acute AIDP that presents with rapidly progressive flaccid weakness
Epidemiology:
• 1-2 cases/100,000 per year
• Slightly greater in males than females
Guillain-Barre Pathophysiology:
• Immune response preceding infection
- Campylobacter jejuni
- HIV, CMV, EBV
- Influenza like illnesses, COVID 19
- Zika virus
- Vaccination (flu, meningococcal, H1N1)
Clinical Manifestations:
• Fairly symmetric muscle weakness
• Absent or depressed DTR’s
• Onset: Few days to a week
• Weakness: mild difficulty with walking to nearly complete paralysis of all extremity, facial, respiratory and bulbar muscles
Guillain-Barre Clinical Features:
• Weakness: Starts in legs and ascends usually. Can begin in arms or face (10% of patients)
• Respiratory Muscle Weakness: May require ventilator support (10-30%)
• Facial Nerve Palsies / Oropharyngeal Weakness
• Oculomotor Weakness
• Decreased / Absent Reflexes: Arms or legs
• Paresthesias: Hands or feet
• Pain: Due to nerve root inflammation. Back or extremities
• Dysautonomia: Life threatening labile BP and arrythmias
• Other: Diarrhea/constipation, hyponatremia, bradycardia, urinary retention, tachycardia, reversible cardiomyopathy, Horner syndrome, Sudden death
Miller Fisher Syndrome:
• Opthaloplegia
• Ataxia
• Areflexia
Guillain-Barre Diagnosis:
• CSF:
- CSF PROTEIN
- NORMAL CSF WBC (Albuminocytologic dissociation)
• Electrodiagnostic studies - EMG, NCV can classify the main variants:
(1) acute polyneuropathy with demyelination features
(2) axonal form
• MRI - Thickening and enhancement of intrathecal spinal nerve roots and cauda equina
• GQ1b IgG Antibody associated with Miller Fisher Variant.
Treatment:
• Plasmapheresis + IVIG
• Mechanical ventilation
• Steroids contraindicated- worse outcome
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