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• acute inflammatory demyelinating polyradiculoneuropathy
• acute motor axonal neuropathy
• acute motor and sensory axonal neuropathy
• Fisher's syndrome
• 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%)

• Campylobacter jejuni infection precedes onset in up to 26-41% of sporadic cases
• viral infection (especially cytomegalovirus and Epstein-Barr virus)
• surgery/trauma

• progressive motor weakness of > 1 limb
• areflexia

Clinical (in order of importance)

• initial 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
• mild sensory symptoms and signs
• facial weakness common (especially in axonal degeneration form) and frequently bilateral. Other cranial nerves may be involved, particularly those innervating tongue, muscles of deglutination and extraocular muscles
• recovery usually start 2-4 weeks after progression stops
• sinus tachycardia, other arrhythmias and labile BP
• absence of fever at onset of neuritic symptoms

• increased CSF protein after 1st week of symptoms or increases on serial examinations

• approx 80% have evidence of nerve conduction slowing or blockage at some point during illness. Neuropathy usually demyelinating but may be axonal

• 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

• 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

• 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

• most prominent form
• segmental loss of myelin in spinal roots and peripheral nerves
• slowing of nerve conduction velocites
• prolongation of distal and F wave latencies
• conduction block
• once immune reactions come to a halt, repair and remyelination set in quickly. Correlates with quick and usually complete recovery.
• In some, particularly those with severe disease inflammatory demyelination is accompanied by variable disruption and loss of axons. Degree of complicating axonal loss is an important determinant of speed of recovery, lasting deficits and ultimately prognosis

• 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.

• 10-20% of sporadic cases
• ¯ /absent distally evoked compound motor action potentials (early sign of denervation) but normal conduction velocities and active potentials in sensory nerves
• predominantly affects motor nerve terminals

• affects nodal regions of oculomotor nerves, dorsal root ganglion cells and cerebellar neurons
• ophthalmoplegia
• ataxia
• areflexia

• 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

• decreases time on a mechanical ventilation
• should be applied early
• 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

• IV pooled gamma globulin (2 g/kg in 2-5 divided 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
• Limited relapses in ~10%. Most respond to repeated course.

Corticosteroids

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

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 ventilatin do not favour use of NIV.

• 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

• 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

• ~75% of patients reach nadir within 2 weeks, 92% within 3 weeks and 94% within 4 weeks
• outcome generally favourable
• 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%

Hughes RAC, Comblath DR. Guillain-Barré syndrome. Lancet, 2005; 366:1653-1666

Hughes RAC et al. Immunotherapy for Guillain-Barré syndrome: a systematic review. Brain, 2007; 130:2245-2257

Jurisdictional Blood Committee, for and on behalf of the Health Minister's Conference. Criteria for the Clinical Use of Intravenous Immunoglobulin in Australia. Canberra: Commonwealth of Australia; 2007

© Charles Gomersall December 1999, January 2009

©Charles Gomersall, May, 2013 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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