Guillain-Barre Syndrome

Diseases, , ,

Guillain-Barre Syndrome (GBS) is an acute inflammatory polyradiculopathy. It is immune mediated and causes ascending weakness in the limbs, respiratory failure and autonomic instability.

Synonyms:

Landry-Guillain-Barre-Strohl syndrome

Clinical features:

  • Progressive ascending weakness in both legs & arms & areflexia
  • Relative symmetry, autonomic dysfunction, mild sensory symptoms

Findings on Investigations:

CSF analysis:

  • Albuminocytological dissociation after one week i.e. raised protein but normal cell count:
  • Protein 100-1000 mg/dL, may be normal initially
  • If WCC is increased consider HIV, Lyme, sarcoid, neoplasia
  • WCC may be increased rarely. [autopsy series] [292]
  • Oligoclonal bands also may occur
  • Myelin basic protein may increase transiently

Nerve conduction studies & EMG:

  • Initially, normal
  • Earliest signs:
  • Loss of an H-reflex, Loss of F-waves
  • CMAP:
    • Demyelination pattern: Temporal dispersion & conduction block, Prolonged distal latencies, decreased conduction velocity
  • Axonal pattern: decreased amplitude
  • EMG: Denervation may be present

 

Subtypes:

  • Acute idiopathic demyelinating polyneuropathy AIDP
  • Acute motor axonal neuropathy AMAN
  • Acute motor sensory axonal neuropathy AMSAN
  • Miller Fisher syndrome MFS: ataxia, ophthalmoplegia, areflexia
  • Pharyngo-cervical brachial PCB variant
  • Bickerstaff Brainstem Encephalitis BBE

Antiganglioside antibodies:

  • Overlap occurs
  • Anti-GQ1b IgG antibody: positive, MFS, BBE, PCB,
  • Anti-GT1a IgG antibody: positive, PCB
  • Anti-GM1, Anti-GM1b, Anti-GD1a, Anti-GalNAc-GD1a: positive, AMAN

Other tests:

  • MRI Lumbar spine with contrast: enhancement of nerve roots

 

Pathology, nerve biopsy:

  • Endoneurium:
    • Inflammation: Infiltrate of macrophages & lymphocytes
  • Immunohistochemsitry: macrophages, CLA for lymphocytes
  • Features more prominent in nerve roots (autopsy specimen)
  • Demyelination occurs
  • Axonal Wallerian degeneration

Treatment:

Monitor the following:

  • Vital signs especially for labile BP
  • Oxygen saturation
  • Bedside forced vital capacity FVC:
    • 20% decrease is a poor prognostic sign
    • <12-15ml/kg is an indication to intubate
  • Negative inspiratory force NIF

Mainly supportive treatment
Admit to ICU/ITU if severely affected
Ventilatory & cardiovascular support as required

  • If intubating, avoid succinylcholine (risk of hyperkalemia)

Supportive care:

  • Passive range of motion to prevent pneumonia & joint contractures
  • Skin care with frequent turning to prevent pressure ulcers
  • DVT prophylaxis
  • Pulmonary toilet and mouth hygiene to prevent pneumonia
  • Turn to prevent decubitus ulcers
  • Pain control

Within 2 weeks of onset of neurologic symptoms:

  • Plasmapharesis (Plasma exchange) or IVIg (within 4 weeks) but not both
  • Start therapy on clinical suspicion before confirmation

Treatment of Dysautonomia:

  • Hypotension:
    • Administer fluids I.V.
    • Pressor drug with extreme caution: Dopamine or phenylephrine
    • Note: Response may be delayed for minutes
  • Hypertension:
    • Short acting beta blockers
    • Note: Hypersensitivity occurs
  • Bradycardia:
    • Sinus tachycardia (due to vagal damage): Fluids I.V.
    • Consider Transvenous pacemaker if non-sinus
  • Adynamic ileus:
    • NG tube +aspirate
    • NPO
  • Atonic bladder:
    • Catheterization

Related articles:

References:

  1. Rauschka, H., et al., Guillain-Barre syndrome with marked pleocytosis or a significant proportion of polymorphonuclear granulocytes in the cerebrospinal fluid: neuropathological investigation of five cases and review of differential diagnoses. Eur J Neurol, 2003. 10(5): p. 479-86.