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• although classically thought of as a disease of the peripheral nervous system, there may be spinal cord changes on autopsy

• Campylobacter jejuni infection precedes onset in up to 26-41% of sporadic cases
• viral infection
• 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
• 10 mononuclear leukocytes/ml

• 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

• 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

• 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

• 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

• IV pooled gamma globulin (0.4 g/kg/day) 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.

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 is completed, at which time disease course can be predicted more easily. Apporximately 1/3 of patients no longer need intubation after 2 weeks

• 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:

© Charles Gomersall December 1999

©Charles Gomersall, May, 2008 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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