Journal of the neurological sciences
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Comparative Study Clinical Trial
Home mechanical ventilation for amyotrophic lateral sclerosis: nasal compared to tracheostomy-intermittent positive pressure ventilation.
People with amyotrophic lateral sclerosis (ALS) usually die from respiratory failure unless they use mechanical ventilation (MV). Many die of respiratory failure without being adequately informed about the available options, such as MV that can provide symptomatic relief and prolong survival. The traditional method of MV used for persons with ALS has been tracheostomy-intermittent positive pressure ventilation (IPPV). ⋯ In conclusion, home mechanical ventilation with nasal or tracheostomy-IPPV are options for selected people with ALS. Nasal-IPPV offers may advantages; it was only used when MV was planned and desired. Nasal-IPPV can be used unless bulbar impairment is severe.
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For the bulbar ALS/MND patient with dysphagia, the accumulation of oral secretions is a distressing problem. The control of these secretions, particularly the thick mucus form, is very difficult. In approaching this problem it is important to realise that the source of these secretions is not just from the oral salivary glands. ⋯ Stimulation of cholinergic receptors produces thin serous secretions whereas beta adrenergic receptors produce thick protein and mucus-rich secretions. Therefore 16 bulbar ALS/MND patients were treated with beta antagonists after maximising other therapy. 75% of this group had fast and significant relief from their thick secretions. It is therefore proposed to perform a large controlled trial of beta blockers in the control of thick oral secretions in ALS/MND based on these promising pilot data.