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Author: Simon Rinaldi

Consider Guillain-Barré syndrome (GBS) (Appendix 71.1) in the patient with progressive symmetrical limb or facial weakness. Management of the patient with suspected GBS is summarized in Figure 71.1.

Priorities

Outline


The priorities are to establish the diagnosis in a timely manner (Tables 71.1 and 71.2; Box 71.1), exclude potential mimics (Table 71.3), and institute appropriate monitoring and therapy to manage or prevent potential complications. The most feared of these are life-threatening – neuromuscular respiratory failure, cardiovascular autonomic disturbance and pulmonary embolism.

Immediate Management!!navigator!!

  • Involve ICU promptly if there is respiratory compromise or autonomic dysfunction.
  • Continue to monitor cardiorespiratory function, including regular spirometry, in progressive phase.
  • Measurement of vital capacity with bedside spirometry is an absolute requirement in the safe management of patients with GBS. Frequent monitoring is required during the progressive phase of the illness.
    • An adult vital capacity of <1.5L/<20 mL/kg is of immediate concern and warrants discussion with ICU as a minimum.
    • At <1L/<15 mL/kg, or with a fall of 50% from baseline on serial testing, prompt ICU involvement is required, and intubation may be necessary.
    • Peak expiratory flow or arterial blood gas measurements are inadequate for assessing neuromuscular respiratory compromise.
  • Treat pain. Paracetamol and opioids can be used, but neuropathic pain agents (e.g. gabapentin, pregabalin, amitriptyline) are often required.
  • Prevent complications: thromboprophylaxis, pressure area care, assess swallow and place nil by mouth (with feeding by nasogastric tube) if compromised.
  • Consider disease modifying treatment: IV immunoglobulin (IVIg) (0.4g/kg×5 days) or plasma exchange (PLEx), in discussion with a neurologist.

Monitoring!!navigator!!

  • Close monitoring of cardiorespiratory function is required initially. As a minimum, two sets of observations an hour apart, including measurement of vital capacity, are suggested following initial presentation. In the rapidly deteriorating patient, even more frequent assessment may be appropriate.
  • Transient arrhythmias (supraventricular tachycardia or bradycardia) without haemodynamic compromise require no treatment. Severe prolonged bradycardia can occur, requiring temporary pacing. Sustained severe hypertension (diastolic BP >120 mmHg) should be treated with labetalol by infusion (p. 349). If hypotension does not respond to IV fluids (guided by measurement of CVP), treat with dopamine or noradrenaline infusion (p. 13).
  • Regular assessment of power can also help to identify whether the disease is in a progressive, plateau or recovery phase.
  • With progressive deterioration, frequent monitoring will need to be maintained, and ICU involved in the event of respiratory compromise, as detailed above. On the other hand, if serial assessments show stability, the frequency of monitoring can be relaxed.
  • In spinal cord pathology, upper motor neuron signs can subsequently develop from what was initially a flaccid paralysis, revealing the true location of the lesion.

Selection of Patients for Ambulatory Care, Hospital Admission and Hdu/Icu Admission!!navigator!!

  • All patients in the progressive phase of the disease will require admission for monitoring as a minimum.
  • Non-ambulant patients and those with cardiovascular autonomic involvement should usually be monitored in a high dependency setting at least.
  • With evidence of neuromuscular respiratory compromise and/or pronounced cardiovascular autonomic instability involvement of ICU is recommended.

Risk-Assessment Models!!navigator!!

  • Risk of mechanical ventilation in first week can be assessed by the Erasmus GBS Respiratory Insufficiency Score (EGRIS, measured at time of admission). Overall, around one in four patients with GBS will require intubation.
  • Long-term prognosis (the probability of being unable to walk independently at four weeks, three months and six months) can be calculated by the modified Erasmus GBS Outcome Score (mEGOS).Both are available via https://gbsstudies.erasmusmc.nl/tools

Further Management

Patients who fail to improve, or continue to deteriorate, after initial treatment with IVIg or PLEx may require a repeat or alternative treatment. There is little evidence to support this decision and discussion with a neurologist is recommended.

For patients in the plateau or recovery phase of their disease, pain management, pressure area care and thromboprophylaxis remain important. Fatigue and low mood are also common problems which may require treatment. Input from physiotherapy is extremely valuable in maintaining and then improving mobility and functional ability. With the aid of occupational therapy, discharge home might be considered at this time, with or without an intermediate period spent in a specialist rehabilitation setting as appropriate.

Patient Support Groups

http://www.gaincharity.org.uk/

http://www.gbs-cidp.org/

Further Reading

Wilson HJ, Jacobs BC, vanDoorn PA (2016) Guillain-Barré syndrome. Lancet 388, 717727.