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Non-invasive ventilation to avoid tracheal intubation in a patient with Guillain-Barré syndrome

2003; Elsevier BV; Volume: 91; Issue: 6 Linguagem: Inglês

10.1093/bja/aeg252

1471-6771

Rupert M. Pearse, Adrian Draper, RM Grounds,

Long-Term Effects of COVID-19

A 72-yr-old man presented with respiratory failure secondary to Guillain-Barré syndrome. Although the criteria for mechanical ventilation were satisfied, the absence of weakness of the bulbar muscles allowed the safe use of non-invasive ventilation for 2 weeks in this patient. Invasive ventilation and tracheostomy were avoided and the patient made a good recovery. A 72-yr-old man presented with respiratory failure secondary to Guillain-Barré syndrome. Although the criteria for mechanical ventilation were satisfied, the absence of weakness of the bulbar muscles allowed the safe use of non-invasive ventilation for 2 weeks in this patient. Invasive ventilation and tracheostomy were avoided and the patient made a good recovery. Respiratory failure in Guillain-Barré syndrome is associated with a poor outcome.1Fletcher DD Lawn ND Wolter TD Wijdicks EF Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation.Neurology. 2000; 54: 2311-2315Crossref PubMed Scopus (143) Google Scholar Up to 30% of patients require invasive mechanical ventilation, often for several weeks.2Schottlender JG Lombardi D Toledo A Otero C Mazia C Menga G Respiratory failure in Guillain-Barré syndrome.Medicina. 1999; 59: 705-709PubMed Google Scholar 3Lawn ND Wijdicks EF Tracheostomy in Guillain-Barre syndrome.Muscle Nerve. 1999; 22: 1058-1062Crossref PubMed Scopus (39) Google Scholar Non-invasive ventilation is a method of administering positive pressure ventilation via a face or nasal mask, and is associated with a lower mortality in other causes of respiratory failure.4Keenan SP Kernerman PD Cook DJ Martin CM McCormack D Sibbald WJ Effect of noninvasive positive pressure ventilation on mortality in patients admitted with acute respiratory failure: a meta-analysis.Crit Care Med. 1997; 25: 1685-1692Crossref PubMed Scopus (261) Google Scholar 5Brochard L Mancebo J Wysocki M et al.Noninvasive ventilation for acute exacerbations of chronic obstructive pulmonary disease.N Engl J Med. 1995; 333: 817-822Crossref PubMed Scopus (1716) Google Scholar We describe the use of non-invasive ventilation in a patient suffering from respiratory failure secondary to Guillain-Barré syndrome. A 72-yr-old male was admitted to hospital with a 2-day history of gradually increasing weakness affecting all limbs, most marked in the distal muscle groups. He also complained of numbness and paraesthesia in his hands and feet. This was preceded by a coryzal illness of 1-week duration for which no medical treatment was sought. Of note was a past medical history of chronic obstructive pulmonary disease and asbestos exposure, for which he was under the care of a respiratory physician. Lung function tests performed 9 months previously documented a vital capacity of 4.22 litre and forced expiratory volume in 1 s (FEV1) of 1.84 litre (89 and 50% predicted, respectively, FEV1/FVC=0.43). There was no evidence of any deterioration in respiratory function before the onset of neurological symptoms. 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 dl−1 (normal range 0.15–0.5 g dl−1), glucose concentration of 3.1 mmol litre−1 and white cell count 3 × 103 mm−3; 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 litre−1. 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 min−1 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 litre−1. 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 10–15 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. Guillain-Barré syndrome or acute inflammatory polyradiculoneuropathy is a syndrome of paralysis, myalgia, and areflexia. Symptoms are usually a result of demyelination, which occurs throughout the peripheral nervous system but is most marked in the nerve roots. In as many as 15% of cases, primary axonal damage results in an acute motor sensory neuropathy, such as the case described.6Govoni V Granieri E Epidemiology of the Guillain-Barre syndrome.Curr Opin Neurol. 2001; 14: 605-613Crossref PubMed Scopus (114) Google Scholar 7Hahn AF Guillain-Barre syndrome.Lancet. 1998; 352: 635-641Abstract Full Text Full Text PDF PubMed Scopus (354) Google Scholar The condition usually occurs after an infective illness. It is likely that immune responses directed towards the infecting organism are involved in the pathogenesis of Guillain-Barré syndrome by cross-reaction with neural tissues.7Hahn AF Guillain-Barre syndrome.Lancet. 1998; 352: 635-641Abstract Full Text Full Text PDF PubMed Scopus (354) Google Scholar Both sensory and motor neurones are affected, but motor involvement is a more prominent feature. Clinical features are of distal paralysis, which progresses to involve proximal muscles including the bulbar and respiratory groups. The myalgia is often severe. 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.6Govoni V Granieri E Epidemiology of the Guillain-Barre syndrome.Curr Opin Neurol. 2001; 14: 605-613Crossref PubMed Scopus (114) Google Scholar Elevated cerebrospinal fluid protein with a normal white cell count occurs in 90% of cases.8Winer JB Hughes RA Anderson MJ Jones DM Kangro H Watkins RP A prospective study of acute idiopathic neuropathy. II. Antecedent events.J Neurol Neurosurg Psychiatry. 1988; 51: 613-618Crossref PubMed Scopus (266) Google Scholar 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.6Govoni V Granieri E Epidemiology of the Guillain-Barre syndrome.Curr Opin Neurol. 2001; 14: 605-613Crossref PubMed Scopus (114) Google Scholar The use of non-invasive ventilation is established in the treatment of acute and chronic respiratory disease,5Brochard L Mancebo J Wysocki M et al.Noninvasive ventilation for acute exacerbations of chronic obstructive pulmonary disease.N Engl J Med. 1995; 333: 817-822Crossref PubMed Scopus (1716) Google Scholar 9Antonelli M Conti G Rocco M et al.A comparison of noninvasive positive-pressure ventilation and conventional mechanical ventilation in patients with acute respiratory failure.N Engl J Med. 1998; 339: 429-435Crossref PubMed Scopus (849) Google Scholar and in chronic neuromuscular respiratory failure.10Shneerson JM Simonds AK Noninvasive ventilation for chest wall and neuromuscular disorders.Eur Respir J. 2002; 20: 480-487Crossref PubMed Scopus (142) Google Scholar 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,11Rabinstein A Wijdicks EF Non-invasive ventilation in acute respiratory failure due to myasthenic crisis may prevent intubation.Neurology. 2002; 59: 1647-1649Crossref PubMed Scopus (97) Google Scholar 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.3Lawn ND Wijdicks EF Tracheostomy in Guillain-Barre syndrome.Muscle Nerve. 1999; 22: 1058-1062Crossref PubMed Scopus (39) Google Scholar 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 kg−l.12Bella IR Chad DA Guillain-Barre syndrome.in: Irwin RS Cerra FB Rippe JM Intensive Care Medicine. Vol. 2. Lippincot-Raven, Philadelphia1999: 2115-2122Google Scholar 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,2Schottlender JG Lombardi D Toledo A Otero C Mazia C Menga G Respiratory failure in Guillain-Barré syndrome.Medicina. 1999; 59: 705-709PubMed Google Scholar 3Lawn ND Wijdicks EF Tracheostomy in Guillain-Barre syndrome.Muscle Nerve. 1999; 22: 1058-1062Crossref PubMed Scopus (39) Google Scholar and is associated with many complications.13Epstein SK Weaning from mechanical ventilation.Respir Care. 2002; 47: 454-468PubMed Google Scholar 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.4Keenan SP Kernerman PD Cook DJ Martin CM McCormack D Sibbald WJ Effect of noninvasive positive pressure ventilation on mortality in patients admitted with acute respiratory failure: a meta-analysis.Crit Care Med. 1997; 25: 1685-1692Crossref PubMed Scopus (261) Google Scholar 5Brochard L Mancebo J Wysocki M et al.Noninvasive ventilation for acute exacerbations of chronic obstructive pulmonary disease.N Engl J Med. 1995; 333: 817-822Crossref PubMed Scopus (1716) Google Scholar 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.7Hahn AF Guillain-Barre syndrome.Lancet. 1998; 352: 635-641Abstract Full Text Full Text PDF PubMed Scopus (354) Google Scholar The mortality in patients ventilated for respiratory failure a result of Guillain-Barré syndrome is 20%.1Fletcher DD Lawn ND Wolter TD Wijdicks EF Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation.Neurology. 2000; 54: 2311-2315Crossref PubMed Scopus (143) Google Scholar This may, in part, be a result of the known complications of mechanical ventilation and tracheostomy.2Schottlender JG Lombardi D Toledo A Otero C Mazia C Menga G Respiratory failure in Guillain-Barré syndrome.Medicina. 1999; 59: 705-709PubMed Google Scholar Experience with this patient suggests that the improved mortality associated with the use of non-invasive ventilation in other causes of respiratory failure,4Keenan SP Kernerman PD Cook DJ Martin CM McCormack D Sibbald WJ Effect of noninvasive positive pressure ventilation on mortality in patients admitted with acute respiratory failure: a meta-analysis.Crit Care Med. 1997; 25: 1685-1692Crossref PubMed Scopus (261) Google Scholar 5Brochard L Mancebo J Wysocki M et al.Noninvasive ventilation for acute exacerbations of chronic obstructive pulmonary disease.N Engl J Med. 1995; 333: 817-822Crossref PubMed Scopus (1716) Google Scholar may also be achieved with Guillain-Barré syndrome.