Chest Dynamic MRI as Early Biomarker of Respiratory Impairment in Amyotrophic Lateral Sclerosis Patients: A Pilot Study.

Background: Amyotrophic lateral sclerosis (ALS) is a neuromuscular progressive disorder characterized by limb and bulbar muscle wasting and weakness. A total of 30% of patients present a bulbar onset, while 70% have a spinal outbreak. Respiratory involvement represents one of the worst prognostic factors, and its early identification is fundamental for the early starting of non-invasive ventilation and for the stratification of patients. Due to the lack of biomarkers of early respiratory impairment, we aimed to evaluate the role of chest dynamic MRI in ALS patients. Methods: We enrolled 15 ALS patients and 11 healthy controls. We assessed the revised ALS functional rating scale, spirometry, and chest dynamic MRI. Data were analyzed by using the Mann-Whitney U test and Cox regression analysis. Results: We observed a statistically significant difference in both respiratory parameters and pulmonary measurements at MRI between ALS patients and healthy controls. Moreover, we found a close relationship between pulmonary measurements at MRI and respiratory parameters, which was statistically significant after multivariate analysis. A sub-group analysis including ALS patients without respiratory symptoms and with normal spirometry values revealed the superiority of chest dynamic MRI measurements in detecting signs of early respiratory impairment. Conclusions: Our data suggest the usefulness of chest dynamic MRI, a fast and economically affordable examination, in the evaluation of early respiratory impairment in ALS patients.

Clinical Characteristics and Outcome of MDR/XDR Bacterial Infections in a Neuromuscular Semi-Intensive/Sub-Intensive Care Unit.

(1) Background: The aim of this study was to assess the clinical and microbiological characteristics of multidrug-resistant infections in a neuromuscular semi-intensive/sub-intensive care unit; (2) Methods: Retrospective analysis on data from 18 patients with NMD with proven MDRO/XDRO colonisation/infection from August 2021 to March 2022 was carried out; (3) Results: Ten patients were males (55.6%), with a median age of 54 years, and there were fourteen patients (77.8%) with amyotrophic lateral sclerosis. All patients had at least one invasive device. Ten (55.6%) patients developed MDRO/XDRO infection (with a median time of 24 days) while six (33.3%) were colonised. The Charlson comorbidity index was >2 in both groups but higher in the infected compared with the colonised (4.5 vs. 3). Infected patients were mostly females (seven patients) with a median age of 62 years. The most common pathogens were Acinetobacter baumannii and Pseudomonas aeruginosa, infecting four (28.6%) patients each. Of eighteen infectious episodes, nine were pneumonia (hospital-acquired in seven cases). Colistin was the most commonly active antibiotic while carbapenems were largely inactive. Eradication of infection occurred in seven infectious episodes (38.9%). None of those with infection died; (4) Conclusions: MDRO/XDRO infections are common in patients with neuromuscular diseases, with carbapenem-resistant non-fermenting Gram-negative bacilli prevailing. These infections were numerically associated with the female sex, greater age, and comorbidities. Both eradication and infection-related mortality appeared low. We highlight the importance of infection prevention in this vulnerable population.

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

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.