A population based study of respiratory function in motor neuron disease patients living in Tayside and North East Fife, Scotland.

Respiratory failure is a major cause of morbidity and the principal cause of death in motor neuron disease; non-invasive ventilation is increasingly used worldwide to palliate the respiratory symptoms. This observational study was designed to evaluate the prevalence of respiratory insufficiency within the motor neuron disease population of Tayside and North East Fife, Scotland. Twenty-six patients were identified, their diagnosis confirmed according to agreed criteria and subjected to the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale, the Epworth Sleepiness questionnaire; spirometry, sniff nasal inspiratory pressure and nocturnal pulse oximetry measurements.Twenty-two (84.6%) patients reported one or more symptoms of respiratory insufficiency, 19 patients (73%) had forced vital capacity <80% of predicted in the sitting position and 10 (38.5%) had oxygen saturation <90% for >5% of night. On this basis a potential 10 patients required consideration for ventilation. As well as probable improvement in quality of life and survival for those patients this potential increase in workload has major educational, management and resource implications for health care providers.

Critical care management of neuromuscular disease, including long-term ventilation.

PURPOSE OF REVIEW: This review highlights recent advances in the critical care management of neuromuscular disease, particularly in the long-term management of chronic respiratory failure occurring as a consequence of neuromuscular disease. RECENT FINDINGS: Although randomized clinical trial evidence of benefit is sparse, a large volume of nonrandomized clinical trial evidence has accumulated demonstrating that noninvasive positive pressure ventilation prolongs and improves quality of life in conditions such as Duchenne muscular dystrophy and motor neuron disease. SUMMARY: Immunomodulatory treatments favorably modify the course of neuromuscular diseases such as Guillain-Barré syndrome, whereas long-term noninvasive positive pressure ventilation has transformed the outlook in previously untreatable conditions such as motor neuron disease and muscular dystrophies. The availability of long-term noninvasive positive pressure ventilation raises major medical, social, economic, and ethical issues that are increasingly being investigated and discussed.

Anti-interleukin-8 autoantibodies in patients at risk for acute respiratory distress syndrome.

OBJECTIVE: To test the hypothesis that elevated concentrations of interleukin-8 associated with anti-interleukin-8 autoantibodies (anti-interleukin-8:interleukin-8 complexes) are found in patients at risk for acute respiratory distress syndrome who developed the disease. DESIGN: Measurement of anti-interleukin-8:interleukin-8 complex concentrations in previously collected bronchoalveolar lavage fluids. These fluids were obtained from patients at risk for acute respiratory distress syndrome who subsequently either recovered or developed acute respiratory distress syndrome. PATIENTS: A unique population of patients at risk for acute respiratory distress syndrome was studied. There were 26 patients at risk for acute respiratory distress syndrome who were divided into three groups. Group I patients had high interleukin-8 concentrations and developed acute respiratory distress syndrome, group II had high interleukin-8 concentrations and did not develop acute respiratory distress syndrome, and group III had low interleukin-8 concentrations and did not develop acute respiratory distress syndrome. These patients were selected to test the hypothesis that presence of elevated concentrations of anti-interleukin-8:interleukin-8 complexes differentiates patients at risk for acute respiratory distress syndrome who developed acute respiratory distress syndrome from patients who did not. MEASUREMENTS AND MAIN RESULTS: Bronchoalveolar lavage fluid concentrations of interleukin-8 associated with the anti-interleukin-8 autoantibodies were significantly different between groups (p <.03). The amount of interleukin-8 bound to the anti-interleukin-8 autoantibody was higher in group I than in group II and group III. CONCLUSIONS: Bronchoalveolar lavage fluid concentration of anti-interleukin-8:interleukin-8 complexes may serve as a marker of disease progression in patients at risk for acute respiratory distress syndrome.