Intensive Care Unit, St James Wing, St Georges Hospital, Blackshaw Road, London SW17 0QT, UK
*Corresponding author. E-mail: rupert.pearse@doctors.net.uk
Accepted for publication: July 9, 2003
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Abstract |
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Br J Anaesth 2003; 91: 913--16
Keywords: complications, Guillain-Barré syndrome; complications, respiratory failure; ventilation, non-invasive positive-pressure
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Introduction |
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Case report |
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Neurological assessment on admission demonstrated areflexia and a symmetrical reduction in power to 4/5 in all limbs (movement possible against gravity and resistance, but weaker than normal). Function of the bulbar muscles was normal and there was no evidence of other cranial nerve dysfunction. Sensory modalities were impaired at the extremities. Cerebrospinal fluid analysis on the day of admission revealed a protein concentration of 0.34 g dl1 (normal range 0.150.5 g dl1), glucose concentration of 3.1 mmol litre1 and white cell count 3 x 103 mm3; no other cells were seen on microscopy. Microbiological culture was negative. Arterial blood gas results were as follows: FIO2 0.21, PaO2 10.3 kPa, PaCO2 4.98 kPa, pH 7.42 and base excess 0.2 mmol litre1. Vital capacity was 2.6 litre and FEV1 l.4 litre (55 and 30% predicted, respectively, FEV1/FVC=0.54).
The patient was admitted to a general medical ward for observation. During the following day muscle power continued to deteriorate, especially in the lower limbs. Diagnoses considered included myasthenia gravis and multiple sclerosis, but in consultation with a neurologist Guillain-Barré syndrome was considered the most likely cause. I.V. immunoglobulin therapy was administered and regular spirometry continued.
On the third day of admission, the vital capacity fell to 0.7 litre (15% predicted) with 1/5 power in all limbs (visible muscle contraction without limb movement). The patient was immediately admitted to the intensive therapy unit (ITU). The patient reported an increase in breathlessness at this time. The ventilatory frequency was 28 min1 with paradoxical abdominal and chest wall motion. Despite clinical deterioration, arterial blood gas results were almost unchanged: FIO2 0.21, PaO2 9.1 kPa, PaCO2 4.1 kPa, pH 7.47 and base excess +0.7 mmol litre1.
The patient was alert, cooperative and maintaining his own airway, with no evidence of difficulties with swallowing or other evidence of bulbar palsy. A trial of non-invasive ventilation was therefore commenced in preference to invasive ventilation (BiPAP Vision, Respironics). Initial ventilatory variables were an inspiratory pressure of 15 cm H2O with an expiratory pressure of 5 cm H2O. Respiratory function stabilized with modest improvement in gas exchange. Vital capacity remained between 0.5 and 1.3 litre. Symptomatically, the patient remained comfortable, alert, and communicative. Regular neurological assessment confirmed adequate bulbar muscle function. With the advice of a speech and language therapist, oral fluids were continued and supplemented by nasogastric feeding. No evidence of autonomic neuropathy was noted and cardiovascular function remained stable. Episodes of sputum retention were treated with chest physiotherapy and tracheal suction via a nasopharyngeal airway, which was placed in the nares intermittently. Ciprofloxacin was given at a dose of 400 mg twice daily for 7 days as empirical antibiotic therapy for a presumed hospital-acquired pneumonia. Microbiological culture of sputum was negative.
Detailed investigation of the cause of the weakness included anti-acetylcholine antibodies, vitamin B12 and folate levels, and thyroid function tests. Microbiological investigations for Campylobacter, infectious mononucleosis, and cytomegalovirus were all negative. The titre for influenza B antigen was significantly elevated, in keeping with recent infection. Electromyographic studies were consistent with an axonal neuropathy and the diagnosis of an acute motor sensory neuropathy, a variant of Guillain-Barré syndrome, was made.
The requirement for non-invasive ventilation was continuous at first; periods of only a few minutes without it resulted in hypoxia and respiratory distress. The ventilatory settings varied little during this period (inspiratory pressure 1015 cm H2O, expiratory pressure 5 cm H2O). Between days 7 and 14, the patient tolerated periods of continuous positive airway pressure and periods breathing oxygen via a high flow oxygen delivery facemask. No problems with mask fit were encountered; pressure areas on the bridge of the nose required an adhesive dressing. The decreasing ventilatory requirement was reflected in a gradual improvement in vital capacity. Power in all limbs improved and the patient was able to stand briefly with the aid of physiotherapy staff.
After 2 weeks in ITU, vital capacity had reached 2.2 litre (46% predicted), the highest value recorded since admission. Discharge to a specialist respiratory ward was arranged and vital capacity monitoring continued on a twice-daily basis. Although tracheal intubation had been considered on more than one occasion, this intervention had been successfully avoided. Further management, which consisted principally of regular physiotherapy, focused on respiratory function and neurological recovery. Repeat electromyographic studies during the third week of admission remained consistent with a diagnosis of the acute motor sensory variant of Guillain-Barré syndrome.
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Discussion |
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Guillain-Barré syndrome is diagnosed on the basis of clinical features with guidance from specific investigations. The criteria for diagnosis remain problematic and continue to evolve with improved understanding of the disease.6 Elevated cerebrospinal fluid protein with a normal white cell count occurs in 90% of cases.8 A preceding infective illness is identified in up to two-thirds of cases and influenza is a recognized antecedent in between 13 and 25% of patients.6
The use of non-invasive ventilation is established in the treatment of acute and chronic respiratory disease,5 9 and in chronic neuromuscular respiratory failure.10 However, non-invasive ventilation is not currently part of the recognized treatment of acute respiratory failure of neuromuscular cause. A potential role in the management of acute myasthenic crises has been suggested,11 but the use of non-invasive ventilation in Guillain-Barré syndrome or any other form of acute respiratory failure of neuromuscular cause has not been described.
Up to 30% of patients with Guillain-Barré syndrome require mechanical ventilation.3 Careful consideration of the risks and benefits of this choice of management is important. Traditionally, invasive ventilation is recommended should vital capacity decrease below 15 ml kgl.12 However, assessment of respiratory function should utilize a range of criteria including symptoms of breathlessness, carbon dioxide retention, bulbar palsy, and paradoxical abdominal wall motion. Invasive ventilation is often required for several weeks,2 3 and is associated with many complications.13 The use of non-invasive ventilation is unlikely to alter the course of the disease in any individual patient, however. But avoidance of the complications of long-term mechanical ventilation in Guillain-Barré syndrome may well be of benefit. This could explain the reductions in mortality associated with the use of non-invasive ventilation in other causes of respiratory failure.4 5
There are potential risks of the use of non-invasive ventilation in respiratory failure secondary to acute neuromuscular disease. The evaluation of the airway and swallowing is of particular importance. Early and continued assessment of the bulbar muscles is required to exclude those patients at risk of aspiration of gastric contents. These assessments were performed daily in our patient to ensure the safe use of non-invasive ventilation. The stomach should also be decompressed on a regular basis as elevated oropharyngeal pressures may result in the swallowing of air. The absence of severe myalgia was an important factor in the safe use of non-invasive ventilation in this patient. The requirement for high doses of opioid analgesia may result in a fluctuating level of consciousness with attendant risks to the airway. Other difficulties with the use of non-invasive ventilation include problems with mask fit and poor patient compliance.
Whilst specific management of Guillain-Barré syndrome with plasma exchange or immunoglobulin has proved effective in limiting the severity and duration of muscle weakness and associated complications, neither has been shown to reduce mortality.7 The mortality in patients ventilated for respiratory failure a result of Guillain-Barré syndrome is 20%.1 This may, in part, be a result of the known complications of mechanical ventilation and tracheostomy.2 Experience with this patient suggests that the improved mortality associated with the use of non-invasive ventilation in other causes of respiratory failure,4 5 may also be achieved with Guillain-Barré syndrome.
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References |
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