phenylephrine-hydrochloride and Amyotrophic-Lateral-Sclerosis

The forced vital capacity (FVC) is an established measure in amyotrophic lateral sclerosis (ALS) clinical trials. Recently the sniff nasal inspiratory pressure (SNIP) test has been increasingly used as a respiratory measure.. It was the aim of this study to assess the feasibility of SNIP as an outcome measure in a phase III clinical trial with a lead-in design.. Twenty patients were enrolled in a randomized clinical trial. FVC, SNIP in sitting (SNIPsitt) and supine (SNIPsup) positions, and the ALS functional rating scale score (ALSFRS-R) were measured every 4 weeks.. Complete data were available for 19 patients over 5 months. Baseline values were normal for FVC (101 ± 14%) but abnormal for SNIPsitt and SNIPsup (84 ± 34% and 82 ± 33%). While FVC and ALSFRS-R declined in parallel, SNIPsitt measures declined significantly less compared to ALSFRS-R (p < 0.05) and FVC (p < 0.001) up to 4 months after enrollment. Over 50% of patients still had values equal to or above baseline SNIPsitt measures after 3 months despite abnormal baseline values.. The delayed decline in SNIP measurements suggests a learning effect over time. The optimal number of SNIPs in ALS clinical trials has yet to be determined. SNIP measures should be used with caution in trials with a lead-in design.

Topics: Adult; Aged; Amyotrophic Lateral Sclerosis; Feasibility Studies; Female; Humans; Inhalation; Male; Middle Aged; Nose; Outcome Assessment, Health Care; Posture; Pressure; Vital Capacity

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). However, the advent of nasal-IPPV has provided a new option for relieving respiratory symptoms and prolonging survival among selected individuals. The ALS Association Data on 75 ALS patients using MV is reviewed. Twenty-five patients used nasal-IPPV, all started electively. Survival with nasal-IPPV ranged from 6 to 64 months for non-bulbar patients, or until the onset of severe bulbar dysfunction when nasal-IPPV no longer was effective. Fifteen of these non-bulbar patients used nasal-IPPV from 20 to 24 h daily; one of these patients used nasal-IPPV continuously for 24 h daily for 24 months. One hundred percent of the users indicated they were glad they chose nasal-IPPV. In contrast, 50 have used tracheostomy-IPPV, usually as a result of emergency hospitalization without advance decision making. Twenty-five patients (50%) lived in a sub-acute skilled nursing facility (SNF) and only 18 of these (72%) were satisfied with their quality of life. Patients using tracheostomy-IPPV with good care are able to live many years: 27 of the 50 (54%) are still living, including one patient who is still living after 14 years of MV. 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.

Topics: Aged; Aged, 80 and over; Amyotrophic Lateral Sclerosis; Caregivers; Female; Home Care Services; Hospices; Humans; Intermittent Positive-Pressure Ventilation; Male; Middle Aged; Nose; Patient Participation; Prospective Studies; Survival Analysis; Tracheostomy; Ventilators, Mechanical

Topics: Abdomen; Adult; Amyotrophic Lateral Sclerosis; Diaphragm; Humans; Inhalation; Lung Transplantation; Nose; Plethysmography; Pressure; Respiratory Function Tests; Respiratory Muscles; Thoracic Wall; Tracheostomy

We investigated two different methodological approaches for determining nasal inspiratory pressure during a sniff (SNIP) in patients with Amyotrophic Lateral Sclerosis (ALS).. We included 37 ALS patients and 11 controls. SNIP was measured in the sitting position, performed in each nostril with (SNIPocclud) and without (SNIPopen) closing the contralateral nostril. The best of 3 consistent results was considered for analyses. Patients were also assessed with MIP and the revised functional ALS rating scale (ALSFRS-R). Mann-Whitney U test, Wilcoxon test and Spearman's rank correlation coefficient with Bonferroni correction were applied. Coefficient of variation (CV) was calculated.. SNIP was significantly lower in patients than controls, either for SNIPopen (p = 0.011) or SNIPocclud (p = 0.002). SNIPopen values were significantly lower both in ALS patients and controls than SNIPocclud (p < 0.001 and p = 0.007, respectively). SNIPopen CV was 8.14% and 8.51%, while SNIPocclud CV was 4.98% and 6.37%, respectively for controls and patients. SNIPopen and SNIPocclud were strongly correlated in both groups (r = 0.761 for controls; r = 0.768 for patients). In ALS, both methods were moderately correlated with MIP (respectively r = 0.525, p = 0.006 and r = 0.685, p < 0.001) and the respiratory subscore of ALSFRS-R (respectively r = 0.525, p = 0.001 and r = 0.64, p < 0.001). Although bulbar and spinal onset patients presented no differences for SNIPopen (p = 0.157), significant differences were found for SNIPocclud (p = 0.018).. SNIPocclud should be considered when evaluating ALS patients as its values present lower variability and favor longer follow-up.

Topics: Adult; Aged; Aged, 80 and over; Amyotrophic Lateral Sclerosis; Female; Humans; Male; Middle Aged; Nose; Pressure; Reproducibility of Results; Respiratory Function Tests; Respiratory Tract Infections

In patients with neuromuscular disorders, no randomized studies have been performed whether nasal or oronasal masks should be preferred. Oronasal masks are often used in acute respiratory failure, while nasal masks are preferred in patients with chronic respiratory failure. However, the use of nasal masks can result in mouth leaks with implications on sleep quality. To reduce these leaks, oronasal masks have been applied during home noninvasive ventilation (NIV). Until now, upper airway obstruction during NIV has been thought to be induced by nasal obstruction, pharyngeal collapse, and/or glottis closure. We report a case indicating another cause of upper airway obstruction: use of an oronasal mask can induce obstructive events in the upper airways, possibly resulting in sleep fragmentation and decreased efficiency of NIV.

Topics: Airway Obstruction; Amyotrophic Lateral Sclerosis; Equipment Design; Humans; Male; Masks; Middle Aged; Mouth; Noninvasive Ventilation; Nose; Polysomnography; Respiratory Insufficiency

Topics: Amyotrophic Lateral Sclerosis; Female; Humans; Inhalation; Male; Nose; Pressure

Topics: Amyotrophic Lateral Sclerosis; Female; Humans; Inhalation; Male; Nose; Pressure

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that rapidly involves the respiratory system, leading to persistent respiratory insufficiency. Neuromuscular respiratory weakness is also responsible for sleep disordered breathing (SDB), which occurs at an early stage of ALS. Maximal sniff nasal-inspiratory pressure (SNIP) is a sensitive test to early disclose respiratory muscle decline. The aim of this study was to evaluate the role of the SNIP test, compared to FVC, as a marker of SDB in ALS. We studied 31 (18 males) patients with ALS, who were divided into two groups according to the SNIP test value. Ten patients who showed a SNIP value higher than 60 cmH(2)O were considered as group 1. Twenty-one patients exhibited a SNIP lower than 60 cmH(2)O and were included in group 2. Both groups of patients were also investigated with nocturnal sleep study. A linear correlation between lower SNIP value and reduced nocturnal SaO(2) in patients with a SNIP value less than 60 cmH(2)O (n = 21; r = 0.449; p = 0.04) was found. A negative correlation between SNIP and time spent in SaO(2) below 90% (TST90) (n = 21; r = -0.584; p = 0.0054), and between SNIP and oxyhaemoglobin desaturation index (ODI, events/hour) (n = 21; r = -0.458; p = 0.0368) was also established in all the patients of group 2, while, in this group, FVC did not correlate with any nocturnal parameter observed. A positive correlation between SNIP and PaO(2) at baseline of the entire population of patients (n = 31; r = 0.614; p < 0.001) was also seen. In conclusion, the results of this preliminary study show that SNIP < 60 cmH(2)O might be considered an early predictor of SDB in ALS.

Topics: Aged; Amyotrophic Lateral Sclerosis; Female; Humans; Inhalation; Male; Middle Aged; Nose; Oxygen; Pressure; Respiratory Function Tests; Sleep Apnea Syndromes

Respiratory muscle weakness is the usual cause of death in amyotrophic lateral sclerosis. The prognostic value of the forced vital capacity (FVC), mouth-inspiratory force, and sniff nasal-inspiratory force were established in a group of 98 patients with amyotrophic lateral sclerosis who were followed trimonthly for 3 years. Sniff nasal-inspiratory force correlated with the transdiaphragmatic pressure (r = 0.9, p < 0.01). Sniff nasal-inspiratory force was most likely to be recorded at the last visit (96% of cases), compared with either the FVC or mouth-inspiratory force (86% and 81%, respectively, p < 0.01). A sniff nasal-inspiratory force less than 40 cm H(2)O was significantly related with nocturnal hypoxemia. When sniff nasal-inspiratory force was less than 40 cm H(2)O, the hazard ratio for death was 9.1 (p = 0.001), and the median survival was 6 +/- 0.3 months. The sensitivity of FVC < 50% for predicting 6-month mortality was 58% with a specificity of 96%, whereas sniff nasal-inspiratory force less than 40 H(2)O had a sensitivity of 97% and a specificity of 79% for death within 6 months. Thus the sniff nasal-inspiratory force test is a good measure of respiratory muscle strength in amyotrophic lateral sclerosis, it can be performed by patients with advanced disease, and it gives prognostic information.

Topics: Adult; Aged; Aged, 80 and over; Amyotrophic Lateral Sclerosis; Female; Follow-Up Studies; Forecasting; Humans; Hypoxia; Inhalation; Male; Middle Aged; Mouth; Nose; Prognosis; Proportional Hazards Models; Respiratory Paralysis; Sensitivity and Specificity; Sleep; Spirometry; Survival Rate; Vital Capacity; Work of Breathing

Topics: Amyotrophic Lateral Sclerosis; Humans; Inhalation; Nose; Prognosis

Patients suffering from amyotrophic lateral sclerosis (ALS) can profit from nasal mechanical ventilation and improve in the quality of life.. Thirty-eight patients were introduced to nasal mechanical ventilation, using pressure- and volume-cycled respirators. The daily periods of mechanical ventilation varied from 8 to 24 hours.. Twelve women and 26 men with ALS mostly with severe symptoms were adapted to the intermittent nasal ventilation. 80% had bulbar symptoms. Nineteen patients died till now. Even when complications occurred it was possible to use the noninvasive ventilation.. Noninvasive mechanical ventilation is possible in spite of complications and improves the quality of life in ALS.

Topics: Adult; Aged; Amyotrophic Lateral Sclerosis; Female; Humans; Male; Middle Aged; Nose; Respiration, Artificial

Previous work confirmed the abnormal potential difference between the undersurface of the inferior nasal turbinate and a reference electrode in cystic fibrosis, but the technique is difficult and the results show overlap between the cystic fibrosis and the control populations. In the present study the potential difference from the floor of the nose has therefore been assessed in normal subjects, as well as in adult patients with cystic fibrosis, bronchiectasis and Young's syndrome. Voltages existing along the floor of the nasal cavity were recorded. The mean potential difference was similar in controls (-18 (SD 5) mv) and in patients with bronchiectasis (-17 (6) mv) and Young's syndrome (-20 (6) mv). The potential difference in cystic fibrosis (-45 (8) mv) was significantly different from controls (p less than 0.002) and there was no overlap between the cystic fibrosis values and values obtained in normal and diseased controls. This simple technique therefore discriminates well between patients with cystic fibrosis and other populations, raising the possibility of its use to assist in diagnosis.

Topics: Adolescent; Adult; Amyotrophic Lateral Sclerosis; Bronchiectasis; Cystic Fibrosis; Female; Humans; Male; Membrane Potentials; Nose; Platybasia; Syndrome