High Risk, Low Prevalence: Guillain-Barré syndrome

Guillain-Barré syndrome is an immune-mediated peripheral neuropathy that classically causes symmetric ascending weakness with hyporeflexia or areflexia. Early bedside recognition matters because respiratory failure and dysautonomia can evolve over days to weeks, even when the first complaint is just distal paresthesias or burning pain.

Bedside recognition of Guillain-Barré

• Classic weakness pattern: GBS usually presents with symmetric ascending weakness beginning in the feet, often after mild distal paresthesias or burning pain rather than major sensory loss.
• Reflex change clue: Hyporeflexia or areflexia is a high-yield bedside finding, with deep tendon reflex changes present in about 90% of patients.
• Recent trigger history: About 75% of patients have an antecedent trigger such as GI illness, URI, vaccination, surgery, pregnancy, or even myocardial infarction, a timeline we get into in the episode.
• Cranial nerve involvement: More than half of patients develop ocular, facial, or bulbar weakness, a pattern that should raise concern for impending airway compromise.
• Miller-Fisher variant: Ophthalmoplegia, ataxia, and areflexia point to Miller-Fisher syndrome, a GBS variant that may have relatively little limb weakness.

Diagnosis and emergency management

• Clinical diagnosis first: GBS remains a clinical diagnosis supported by testing, while labs and imaging help exclude mimics such as hypokalemia, hypophosphatemia, and spinal cord disease.
• CSF protein pattern: Albuminocytologic dissociation with elevated CSF protein and a normal cell count supports GBS, but it is often absent in the first few days. We cover when that early negative LP should not reassure you in the chapter.
• Progression and symmetry: Weakness that worsens over days to weeks, stays symmetric, and lacks severe sensory deficits fits GBS better than stroke, myelopathy, or chronic neuropathy.
• Autonomic instability risk: About two-thirds of patients develop dysautonomia with blood pressure swings or tachyarrhythmias, and only persistent abnormalities generally merit treatment.
• Airway warning signs: Bulbar weakness, poor secretion handling, weak cough, and an abnormal single-breath count are practical red flags for respiratory decompensation.
• Definitive treatment options: IVIG and plasma exchange are the main disease-directed therapies and appear similarly effective, while combining them does not improve outcomes.

References:

1. Madden J, Spadaro A, Koyfman A, Long B. High risk and low prevalence diseases: Guillain-Barré syndrome. Am J Emerg Med. 2024 Jan;75:90-97. Epub 2023 Oct 28. PMID: 37925758