Which are the factors influencing NIV adaptation and tolerance in ALS patients?
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ORIGINAL ARTICLE
Which are the factors influencing NIV adaptation and tolerance in ALS patients? Massimo Russo 1 & Carmen Bonanno 1 & Claudia Profazio 2 & Stefania La Foresta 2 & Cristina Faraone 2 & Andrea Lizio 3 & Gian Luca Vita 2 & Maria Sframeli 2 & Irene Aricò 2 & Paolo Ruggeri 4 & Antonio Toscano 1 & Giuseppe Vita 1,2 & Christian Lunetta 3 & Sonia Messina 1,2 Received: 10 June 2020 / Accepted: 20 July 2020 # Fondazione Società Italiana di Neurologia 2020
Abstract Amyotrophic lateral sclerosis (ALS) is a multisystemic disease compromising both the neuromuscular system and the cognitive status. Non-invasive ventilation (NIV) has been shown to improve survival and quality of life in ALS patients with respiratory failure, but scanty literature investigated which are the predictors of NIV tolerance. The aim of this study was to evaluate the impact of functional, cognitive, neurobehavioral, and respiratory status on NIV compliance and tolerance in patients with ALS. We retrospectively evaluated clinical data of ALS patients who consecutively underwent a NIV trial during hospitalization. Cognitive and neurobehavioral assessments have been performed using the Edinburgh Cognitive and Behavioral ALS Screen (ECAS), the Hospital Anxiety and Depression Scale (HADS), the Frontal Assessment Battery (FAB), the Raven’s 47 Colored Progressive Matrices (PM47), and the Neurobehavioral Rating Scale Revised (NRSR). Seventy-two patients (mean age ± SD; 63.9 ± 10.6 years) were included. Patients adapted were 63/72 (87.5%). The average time of adaptation was 7.82 ± 5.27 days. The time required to reach a satisfying NIV adaptation was significantly related to the presence of sialorrhea (p = 0.02), respiratory status (Borg Dyspnoea Scale, p = 0.006, and ALS-FRS-R respiratory subscore, p = 0.03) and behavioral and cognitive impairment (NRSR-F1, p = 0.04, NRSR- F5, p = 0.04). Presence of sialorrhea and neurobehavioral impairment, and absence of respiratory symptoms are negative predictors of NIV adaptation. This study highlights the need of a multidisciplinary patienttailored approach including cognitive-behavioral assessment and a psychological support program to optimize patient’s training and compliance to NIV. Keywords Amyotrophic lateral sclerosis . Non-invasive ventilation . Compliance . Neurobehavioral status . Multidisciplinary setting
Introduction
* Giuseppe Vita [email protected] 1
Unit of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy
2
Nemo Sud Clinical Center for Neuromuscular Disorders, University Hospital “G. Martino”, Messina, Italy
3
NEuroMuscular Omnicenter, Serena Onlus Foundation, Milan, Italy
4
Pulmonology Unit, Department of Biomedical and Dental Sciences, Morphological and Functional Images, University of Messina, Messina, Italy
Amyotrophic lateral sclerosis (ALS) is a heterogeneous neurodegenerative rare disease of adult life, leading to severe weakness of bulbar, limb, and respiratory muscles and consequent short life expect
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