Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
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Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Apr 2003
Clinical Trial Controlled Clinical TrialNocturnal pulse oximetry: a new approach to establish the appropriate time for non-invasive ventilation in ALS patients.
Previous studies have supported non-invasive ventilation (NIV) in amyotrophic lateral sclerosis (ALS), indicating that it prolongs survival. However, criteria for the use of NIV are yet to be defined. The aim of the present study was to evaluate the potential use of nocturnal pulse oximetry (NPO) as a tool for determining the most appropriate time at which to initiate NIV in ALS patients. ⋯ Survival was longer in Group 2 (p<0.002). We concluded that NPO is a valuable screening test to establish the need for NIV. Our results also suggest that the early diagnosis of RI and the early use of NIV increase patient compliance with NIV.
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Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Apr 2003
Case ReportsExpiratory muscle weakness and assisted cough in ALS.
Elimination of airway secretion is a major issue in the care of patients with ALS. Sufficient cough flows have to be generated by expiratory muscles to allow airway clearance. ⋯ Expiratory aids may help to overcome these problems, at least for some time. We report a patient with advanced ALS, receiving nocturnal NIV, who gained much benefit from regular use of a mechanical in-exsufflation device.
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Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Sep 2002
Non-invasive ventilation in motor neuron disease: current UK practice.
To evaluate the clinical application of non-invasive ventilation (NIV) in motor neuron disease (MND) in the UK. ⋯ In the UK, few patients with MND are treated with NIV. There is marked variation in clinical practice. This may reflect uncertainty about the role of non-invasive ventilation in MND, and emphasizes the need for a randomized controlled trial to assess the impact of NIV on quality of life and survival.
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Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Jun 2002
Cu/Zn superoxide dismutase (SOD1) mutations associated with familial amyotrophic lateral sclerosis (ALS) affect cellular free radical release in the presence of oxidative stress.
The exact molecular mechanisms by which mutations in Cu/Zn superoxide dismutase (SOD1) cause motor neuron injury remain incompletely understood, though a body of evidence suggests that the mutant protein exerts a cell-specific toxic gain of function. The role of nitric oxide (NO) in SOD1-related motor neuron injury has been particularly controversial. Theoretically, there are arguments to suggest that NO may exert an important role in motor neuron injury, but there is relatively little direct experimental support for this hypothesis. ⋯ NO is likely to contribute to motor neuron injury, but this does not fully account for all the cellular toxic effects of mutant SOD1.
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Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Mar 2002
ReviewAmyotrophic lateral sclerosis: evaluation and treatment of respiratory impairment.
Patients with amyotrophic lateral sclerosis (ALS) invariably develop respiratory muscle weakness and most die from pulmonary complications. There are numerous tests available to evaluate respiratory status in ALS and it is important to understand their various advantages and limitations. Forced vital capacity (FVC) is commonly used but can remain normal despite substantial inspiratory muscle weakness. ⋯ Observational studies have demonstrated improved survival and quality of life with noninvasive ventilation. Tracheostomy with long-term mechanical ventilation is not frequently used but can be an important component of care for ALS. This review describes an approach to respiratory evaluation and care of patients with ALS.