Anna Cassano

Early treatment with noninvasive positive pressure ventilation prolongs survival in Amyotrophic Lateral Sclerosis patients with nocturnal respiratory insufficiency

BackgroundAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, which rapidly leads to chronic respiratory failure requiring mechanical ventilation. Currently, forced vital capacity (FVC) < 50% is considered as physiologic marker for admitting patients to Noninvasive Positive Pressure Ventilation (NPPV) intervention, although it has been recently shown the median survival of patients with baseline FVC < 75% much shorter than median survival of patients with baseline FVC > 75%, independently by any treatment.AimTo assess the role of NPPV in improving outcome of ALS, a retrospective analysis was performed to investigate 1 year survival of ALS patients with FVC < 75% and nocturnal respiratory insufficiency, treated with NPPV, compared to a well-matched population of ALS patients, who refused or was intolerant to NPPV.MethodsWe investigated seventy-two consecutive ALS patients who underwent pulmonary function test. Forty-four presented a FVC > 75% and served as control group. Twenty-eight patients presented a FVC < 75% and showed, at polysomnography analysis, nocturnal respiratory insufficiency, requiring NPPV; sixteen were treated with NPPV, while twelve refused or were intolerant.ResultsIncreased survival rate at 1 year in patients with FVC < 75% treated with NPPV, as compared to those who refused or could not tolerate NPPV (p = 0.02), was observed. The median rate of decline in FVC% was slower in NPPV patients than in patients who did not use NPPV (95% CI: 0.72 to 1.85; p < 0.0001).ConclusionThis report demonstrates that early treatment with NPPV prolongs survival and reduces decline of FVC% in ALS.

Association between low sniff nasal-inspiratory pressure (SNIP) and sleep disordered breathing in amyotrophic lateral sclerosis: Preliminary results

Abstract 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 cmH2O were considered as group 1. Twenty-one patients exhibited a SNIP lower than 60 cmH2O 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 SaO2 in patients with a SNIP value less than 60 cmH2O (n = 21; r = 0.449; p = 0.04) was found. A negative correlation between SNIP and time spent in SaO2 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 PaO2 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 cmH2O might be considered an early predictor of SDB in ALS.

The Prognostic Role of Obstructive Sleep Apnea at the Onset of Amyotrophic Lateral Sclerosis

Background/Objective: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the progressive loss of central and peripheral motor neurons. Some studies have found discordant data in the presence of sleep apnea in ALS patients. An obstructive component also occurs with upper airways hypotonia and muscle weakness that may result in an excessive reduction of airway lumen, leading to obstructive sleep apnea (OSA). The aim of this study was to assess the role of obstructive apneic events at disease onset in the ALS prognosis. Methods: A longitudinal retrospective study was conducted on 42 clinically diagnosed ALS patients. The study population was divided into 2 groups according to their obstructive apnea/hypopnea index (AHIo): group 1 consisted of 20 patients with an AHIo ≥5 and group 2 consisted of 22 patients with an AHIo <5. Both groups were compared with regard to demographic, polygraphic, and respiratory function parameters as well as ALS characteristics (bulbar onset, time between onset and first check-up, time between diagnosis and first check-up, time between first check-up and death or tracheostomy). Results: The mean survival in ALS patients with an AHIo ≥5 was significantly shorter than in ALS without OSA (p = 0.0237). The sniff nasal inspiratory pressure test was significantly correlated with AHIo, time of oxyhemoglobin saturation below 90% and the oxyhemoglobin desaturation index (p < 0.0001). Conclusions: Our study highlights the importance of an early diagnosis of OSA in ALS patients, allowing the identification of ALS patients with an OSA phenotype (AHIo ≥5), who are characterized by a worse prognosis.