#71 – Guillain-Barré Syndrome



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DEFINITION

  • Acute monophasic paralyzing illness usually provoked by a preceding event
    • Most commonly this is an infection (2/3rds of patients)
    • Other causes
      • Immunizations
        • Influenza, meningococcal (MCV4)
      • Surgery
      • Trauma
      • Medications
  • Was considered to be a SINGLE condition, but now is recognized to be an umbrella term for a mix of many different variants
    • Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
      • Most common in the US (85-90%)
    • Miller Fisher syndrome (MFS)
    • Acute motor axonal neuropathy (AMAN)
    • Acute sensorimotor axonal neuropathy (AMSAN)

EPIDEMIOLOGY

  • 1-2 cases per 100,000 per year
  • Incidence increases 20% with every 10-year increase in age after the first decade of life
  • Slightly higher incidence in men than women

PATHOGENSIS

  • Molecular Mimicry
    • Antecedent event provokes an immune response, which produces antibodies that cross-reacts with nerve components due to the sharing of epitopes
      • If directed at Schwann cell surface membranes à AIDP
        • Infiltration of lymphocytes in the epineural and endoneural veins causing myelin degeneration at the dorsal and ventral roots
      • If directed at axonal membrane à AMAN and AMSAN
        • Attack is directed at the nodes of Ranvier of the ventral roots of the peripheral nerves
    • End result is blocking the neuro-electrical transmission of impulses
  • Common infections
    • C. jejuni (most common)
    • HIV
    • H. influenza
    • M. pneumoniae
    • CMV

CLINICAL FEATURES

  • Ascending (distal to proximal) symmetric muscle weakness with absent deep tendon reflexes progressing over days to weeks
    • Weakness can vary from mild difficulty with walking to complete paralysis
    • Symptom peak at around 4 weeks
  • Respiratory dysfunction – 10-30%
  • Facial nerve palsies or oropharyngeal weakness – 50%
  • Oculomotor – 15%
  • Paresthesias – 80%
  • Dysautonomia – 70%
    • Diarrhea, constipation, hyponatremia, bradycardia, urinary retention
  • Variant Discrepancies
    • AMAN – preservation of deep tendon reflexes
    • AMSAN – more sensory disturbances
    • Miller Fisher – ophthalmoplegia with ataxia and areflexia

DIAGNOSIS

  • CSF Analysis
    • Albuminocytologic dissociation
      • Elevated protein with normal WBC
      • Present within first week and peaks at 3 weeks
      • Thought to be due to the increased permeability of the blood-nerve-barrier of the proximal roots
  • Electrodiagnostic Studies (EMG and NCS)
    • Demyelinating forms
      • Decreased motor nerve conduction velocity
      • Prolonged distal motor latency
      • Complete conduction blocks
    • Axonal forms
      • Decreased distal motor and/or sensory amplitudes
    • Serial studies can be helpful since the condition can progress over time
  • Antibodies
    • Specific autoantibodies can help differentiate variant subtypes
  • MRI
    • Thickening and enhancement of the intrathecal spinal nerve roots and cauda equina

DIAGNOSTIC CRITERIA

  • Developed in 1978 and modified over the decades based on evolving research

TREATMENT

  • Supportive care
    • Respiratory support
      • Up to 30% will require ventilatory support
      • Impending respiratory arrest
        • FVC < 20 mL/kg
        • Maximum inspiratory pressure < 30 cmH2O
        • Maximum expiratory pressure < 40 cmH2O
      • Predictors of respiratory failure
        • Time of onset to admission < 7 days
        • Inability to cough, stand, lift elbows, or lift head
        • Elevated LFTs
        • Vital capacity < 60% predicted
    • Autonomic Dysfunction
      • Cardiovascular monitoring and support
        • Hypertension and hypotension
        • Bradycardia and tachydysrhythmias
      • Bowel and Bladder care
        • Urinary retention
        • Ileus management
  • Disease-Modifying Treatment
    • Started within 4 weeks of symptom onset
    • Plasma exchange
      • Remove circulating autoantibodies
      • 4-6 treatments over 8-10 days
      • Complications
        • Hypotension, sepsis
    • IVIG
      • Interferes with production and function of cytokines, B-cells, and T-cells
      • 0.4 grams/kg/day for 5 days
      • Complications
        • Meningitis, rash, renal failure

PROGNOSIS

  • 60% will fully recover by 1 year
  • 15% will have persistent severe motor problems
  • 3-7% will succumb to the illness
    • 20% of patients requiring mechanical ventilation

COTTAGE PHYSICIAN (1893)



REFERENCES

  1. Gibbons CH, Engstrom JW. Disorders of the Autonomic Nervous System. In: Jameson J, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 20e. McGraw-Hill; Accessed May 09, 2021.
  2. Diseases of the Peripheral Nerves. In: Ropper AH, Samuels MA, Klein JP, Prasad S. eds. Adams and Victor’s Principles of Neurology, 11e. McGraw-Hill; Accessed May 09, 2021.
  3. Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med. 2012; 366(24):2294-304. [pubmed]
  4. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016; 388(10045):717-27. [pubmed]
  5. Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology. 2011; 36(2):123-33. [PDF]
  6. Alshekhlee A, Hussain Z, Sultan B, Katirji B. Guillain-Barré syndrome: incidence and mortality rates in US hospitals. Neurology. 2008; 70(18):1608-13. [pubmed]
  7. Fokke C, van den Berg B, Drenthen J, Walgaard C, van Doorn PA, Jacobs BC. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain. 2014; 137(Pt 1):33-43. [pubmed]
  8. Hadden RD, Cornblath DR, Hughes RA, et al. Electrophysiological classification of Guillain-Barré syndrome: clinical associations and outcome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Ann Neurol. 1998; 44(5):780-8. [pubmed]
  9. Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EF. Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol. 2001; 58(6):893-8. [pubmed]
  10. Raphaël JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2012; [pubmed]
  11. Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2014; [PDF]

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