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Guillain Barre syndrome

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Guillain-Barré syndrome

Last updated February 2015 by Charles Gomersall

Types

  • acute inflammatory demyelinating polyradiculoneuropathy
  • acute motor axonal neuropathy
  • acute motor and sensory axonal neuropathy
    • Fulminant onset of severe paralysis and sensory deficits
    • Acute axonal degeneration
    • ¯¯¯ or absent evoked responses on distal supramaximal stimulation of motor and sensory nerves. Progresses rapidly to total loss of electrical excitability.
  • Miller-Fisher syndrome
    • affects nodal regions of oculomotor nerves, dorsal root ganglion cells and cerebellar neurons
    • ophthalmoplegia
    • ataxia
    • areflexia
  • Overlap syndromes

In North America and Europe only 5% of cases have axonal types, which are much more common in China, Japan and Central and South America (~30-45%)

Precipitating factors

  • Campylobacter jejuni infection precedes onset in up to 26-41% of sporadic cases. Associated with acute motor axonal neuropathy subtype
  • viral infection (especially cytomegalovirus and Epstein-Barr virus)
  • surgery/trauma

Pathogenesis

  • Immune response to infectious disease generates antibodies with cross-react with gangliosides at nerve membranes resulting in nerve damage or functional blockage or nerve conduction
  • Type of preceding infection and specificity of antiganglioside antibodies largely determine subtype and clinical course

Diagnostic criteria

Features required for diagnosis

  • progressive motor weakness of > 1 limb
  • areflexia or hyporeflexia

Features supportive of the diagnosis

Clinical

  • Onitial rapid progression of weakness but no further progression by 4 weeks
  • Symmetry seldom absolute but if one limb is affected opposite one is likely to be affected
  • Autonomic dysfunction

    Acute motor axonal neuropathy

  • No sensory symptoms or signs

  • Cranial nerve involvement (rarely)

  • Pain (sometimes)

    Acute inflammatory demyelinating polyneuropathy

  • Mild sensory symptoms or signs

  • Cranial nerve involvement, especially bilateral weakness of facial muscles

  • Pain (often)

Nerve conduction studies

    Acute motor axonal neuropathy

  • No features of demyelination
  • Distal compound muscle action potential <80% lower limit of normal in at least 2 nerves
  • +/- transient motor nerve conduction block

    Acute inflammatory demyelinating polyneuropathy

  • Features of demyelination
  • Prolonged distal motor latency
  • Decreased motor nerve conduction velocity
  • Increased F-wave latency, conduction blocks and temporal dispersion

Features that should raise doubt about diagnosis

  • Increased mononuclear cells in CSF (>50 per microlitre)
  • Polymorphonuclear cells in CSF
  • Severe pulmonary dysfunction with limited limb weakness at onset
  • Severe sensory signs with limited limb weakness at onset
  • Bladder or bowel dysfunction at onset or persistent dysfunction
  • Fever at onset
  • Sharp spinal cord sensory level
  • Slow progression with limited weakness and without respiratory involvement
  • Markedly asymmetrical weakness

Intensive care issues

Respiratory dysfunction

  • impaired expiratory effort (cough)
  • weakness of tongue and retropharyngeal muscles causes positional airway obstruction
  • inspiratory muscle weakness
  • increased risk of aspiration due to weakness of laryngeal and glottic muscles
  • ventilatory drive and CO2 response generally normal

Assessment of respiratory function

  • respiratory dysfunction may be compromised before clinical signs are clinically obvious
  • ventilatory muscle insufficiency may not correlate well with general neuromuscular examination
  • serial assessment is essential to ensure prompt transfer of patients to ICU at the onset of ventilatory failure
  • respiratory rate
  • paradoxical abdominal movement
  • frequent changes in breathing pattern to alternate between major and accessory respiratory muscles
  • use of accessory muscles during quiet breathing
  • cough
  • ability to protect airway: nasal speech, difficulty with protruding tongue and difficulty swallowing indicate that bulbar involvement is significant and ventilatory failure is imminent
  • presence of atelectasis or pulmonary infiltrates on CXR suggest rapidly evolving ventilatory failure
  • maximum inspiratory force < 20 cm H2O associated with high risk of ventilatory insufficiency
  • FVC < 15 ml/kg associated with increased risk of ventilatory failure, < 10 indicative of ventilatory failure
  • ABG: changes occur late

Autonomic dysfunction

  • common in demyelinating form but relatively uncommon in axonal types
  • cardiac arrhythmias
  • labile BP
  • abnormal haemodynamic response to drugs
  • ECG abnormalities
  • pupillary dysfunction
  • sweating abnormalities
  • urinary retention
  • GI dysfunction
  • blood volume and electrolyte disturbances are common due to loss of autonomic control of systemic blood flow distribution, renin and aldosterone release and fluid loss from sweat glands

Investigations

  • CSF examination
    • before treatment with IV Ig, which may cause aseptic meningitis
  • Nerve conduction studies
  • ± stool culture and serology for C. jejuni
  • ± acute and convalescent serology for CMV, EBV and M. pneumoniae as a minimum
  • ± antibodies to gangliosides GM1, GD1a and GQ1b

     
    Type Antibodies
    Acute inflammatory demyelinating polyradiculopathy Unknown
    Acute motor and sensory axonal neuropathy GM1, GM1b, GDa1
    Acute motor axonal neuropathy GM1, GM1b, GDa1, GalNac-GD1a
    Fisher's syndrome GQ1b, GT1a

Differential diagnosis

Causes of acute flaccid paralysis

Treatment

Plasmapheresis

  • decreases time on a mechanical ventilation if given within 2 weeks of onset
  • 1.5-2 plasma volumes on alternate days. 2 treatments for those with mild disease and 4 for those with moderate or severe disease
  • early completion of plasmapheresis may be followed by relapses, which may respond to further plasma exchange
  • no evidence that routine administration of IV Ig after plasmapheresis improves outcome

Immunoglobulin

  • IV pooled gamma globulin (2 g/kg in 2-5 divided daily doses) given in the first 2 weeks of the disease is as effective as plasmapheresis.
  • IV IgG should now be treatment of choice for patients with severe GBS during the first 2 weeks after start of neuropathic symptoms on grounds of equivalent efficacy, similar cost, greater convenience and ease of administration. However there are some data to suggest that patients with acute motor axonal neuropathy respond better to plasmpheresis than immunoglobulin.
  • Limited relapses in ~10%. Most respond to repeated course.

Corticosteroids

  • No evidence of benefit and may actually result in worse outcome

Supportive

General: DVT prophylaxis, good neuro and chest physio, psychological support, communication aids

Respiratory

  • some evidence that early intubation and ventilation reduces pulmonary complications
  • objective criteria for ventilation include FVC < 10 ml/kg and maximum inspiratory force < 30 cm H2O. Do not rely on ABGs. May require intubation at FVC <15 ml/kg due to inability to clear secretions
  • decision with regard to tracheostomy may be best left until after plasmapheresis or IV Ig is completed, at which time disease course can be predicted more easily. Approximately 1/3 of patients no longer need intubation after 2 weeks
  • little data to support use of NIV. Inability to cough and requirement for relatively long period of ventilation do not favour use of NIV.

Cardiac

  • symptomatic bradycardia, complete heart block and cardiac arrest are indications for insertion of temporary pacing wire
  • caution with vasodilators and anaesthetics in view of inability to increase cardiac output in response to vasodilatation

Pain

  • may be related to pressure areas and may be partially relieved by sheepskins and frequent turning
  • socks and warming devices may give some relief
  • NSAIDs may help joint aches and pains but opioids usually necessary
  • antidepressants may be used as an adjuvant to manage sleep disturbance, paraesthetic pain and emotional consequences of illness
  • epidural opioids may be useful in controlling pain unresponsive to conventional methods

Prognosis

  • ~75% of patients reach nadir within 2 weeks, 92% within 3 weeks and 94% within 4 weeks
  • outcome generally favourable but 20% of patients are unable to walk unaided at 6 months
  • most reliable indicators of significant residual disability at 1 year:
  • age >60 yrs (most consistent finding)
  • progression to quadraplegia in <1 week
  • mean distal motor amplitude <20% of lower limit of normal
  • preceding diarrhoeal illness
  • most patients with acute motor axonal neuropathy have good otucome:
    • 86% able to walk independently at 6 months
    • all able to walk eventually
  • mortality 5-8%

Further reading

van den Berg B, Walgaard C, Drenthen J et al. Nat Rev Neurol, 2014; 10:469-82


© Charles Gomersall December 1999, January 2009

 

©Charles Gomersall, February, 2015 unless otherwise stated. The author, editor and The Chinese University of Hong Kong take no responsibility for any adverse event resulting from the use of this webpage.
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