{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T01:44:03Z","timestamp":1760060643492,"version":"build-2065373602"},"reference-count":19,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2025,9,6]],"date-time":"2025-09-06T00:00:00Z","timestamp":1757116800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>Introduction: Myotonic dystrophies (DM) are progressive genetic disorders with multisystemic involvement, particularly affecting the muscular, respiratory, and neuropsychological systems. Myotonic dystrophy type 1 (DM1), or Steinert\u2019s disease, may lead to severe respiratory complications, including sleep-disordered breathing and hypercapnia, often requiring noninvasive ventilation to manage respiratory failure. However, adherence to NIV remains a major challenge, often influenced by cognitive and psychological factors such as apathy and depression. This study aims to investigate the presence of depression and SDB in patients with DM1 initiating NIV, and to evaluate factors influencing adherence to ventilatory support. Materials and Methods: We selected 13 adult patients (\u226518 years) with diagnosis of Steinert\u2019s disease with respiratory impairment who needed to start respiratory support. Dysphagia was assessed in all patients at baseline by a videofluoroscopic swallow study. Beck\u2019s Depression Inventory II was administered for measuring the severity of depression. The Montreal Cognitive Assessment was used as a screening tool to detect signs of neurocognitive disorders. We evaluated adherence to NIV. Results: The study population presented with sleep-disordered breathing, as indicated by a median apnea\u2013hypopnea index (AHI) of 24 events per hour (IQR: 14.2\u201334.5) and an oxygen desaturation index (ODI) of 25 events per hour (IQR: 18\u201333). Adherence to NIV was obtained in seven patients. No difference in baseline lung function was observed. Adherent subjects had moderate hypercapnia at baseline; pCO2 was 52 vs. 49 mmHg. Non-adherent patients showed a higher prevalence of depression with a median BDI-II score of 18 vs. 6 in adherent patients. The findings highlight that psychological factors, especially depression, play a crucial role in adherence to NIV. Interestingly, depression was not linked to initial respiratory measurements but showed a significant association with nocturnal oxygen desaturation. This suggests that relying solely on clinical and respiratory assessments may not be adequate to predict or improve treatment adherence. Conclusions: Incorporating psychological evaluations and addressing mental health issues, such as depression, are essential steps to enhance NIV compliance and overall DM1 patient outcomes. A multidisciplinary approach combining respiratory and psycho-emotional interventions is crucial for effective disease management.<\/jats:p>","DOI":"10.3390\/brainsci15090968","type":"journal-article","created":{"date-parts":[[2025,9,10]],"date-time":"2025-09-10T12:04:55Z","timestamp":1757505895000},"page":"968","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Adherence to Non-Invasive Ventilation in Steinert Disease: Clinical and Psychological Insights"],"prefix":"10.3390","volume":"15","author":[{"given":"Anna","family":"Annunziata","sequence":"first","affiliation":[{"name":"Unit of Respiratory Pathophysiology, Monaldi-Cotugno Hospital, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9457-6558","authenticated-orcid":false,"given":"Francesca","family":"Simioli","sequence":"additional","affiliation":[{"name":"Unit of Respiratory Pathophysiology, Monaldi-Cotugno Hospital, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2367-6604","authenticated-orcid":false,"given":"Giorgio Emanuele","family":"Polistina","sequence":"additional","affiliation":[{"name":"Unit of Respiratory Pathophysiology, Monaldi-Cotugno Hospital, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8589-0529","authenticated-orcid":false,"given":"Anna Michela","family":"Gaeta","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Hospital Universitario Severo Ochoa, Av. de Orellana, s\/n, 28914 Legan\u00e9s, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-5012-6525","authenticated-orcid":false,"given":"Maria","family":"Cardone","sequence":"additional","affiliation":[{"name":"Unit of Respiratory Pathophysiology, Monaldi-Cotugno Hospital, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5232-3678","authenticated-orcid":false,"given":"Camilla","family":"Di Somma","sequence":"additional","affiliation":[{"name":"Unit of Respiratory Pathophysiology, Monaldi-Cotugno Hospital, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raffaella","family":"Manzo","sequence":"additional","affiliation":[{"name":"Unit of Respiratory Pathophysiology, Monaldi-Cotugno Hospital, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonella","family":"Marotta","sequence":"additional","affiliation":[{"name":"Unit of Respiratory Pathophysiology, Monaldi-Cotugno Hospital, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4293-4461","authenticated-orcid":false,"given":"Cecilia","family":"Calabrese","sequence":"additional","affiliation":[{"name":"Department of Translational Medical Sciences, University of Campania \u201cLuigi Vanvitelli\u201d, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2523-9769","authenticated-orcid":false,"given":"Giuseppe","family":"Fiorentino","sequence":"additional","affiliation":[{"name":"Unit of Respiratory Pathophysiology, Monaldi-Cotugno Hospital, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,9,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1136\/jnnp.2008.158261","article-title":"The myotonic dystrophies: Diagnosis and management","volume":"81","author":"Turner","year":"2010","journal-title":"J. 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