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:
- 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.