{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T05:57:59Z","timestamp":1785477479775,"version":"3.56.0"},"reference-count":53,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T00:00:00Z","timestamp":1785369600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T00:00:00Z","timestamp":1785369600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100032701","name":"Bayerisches Staatsministerium f\u00fcr Gesundheit, Pflege und Pr\u00e4vention","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100032701","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neurol. Res. Pract."],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>In advanced amyotrophic lateral sclerosis (ALS), eye movements often represent the last channel for intentional communication. While oculomotor function has traditionally been considered relatively preserved, emerging evidence indicates progressive impairment in long-term survivors on tracheostomy-invasive ventilation (TIV). As a result, eye-based communication may become increasingly unreliable before complete loss, challenging clinical decision-making and advance care planning (ACP).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a case-based topical review integrating clinical observation and literature to examine the trajectory of oculomotor decline, its impact on communication, and implications for treatment decisions. Three patients with ALS receiving TIV in a home-care setting illustrate key clinical and ethical challenges.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Across cases and literature, oculomotor decline followed a gradual trajectory from effective eye-tracking communication to a complete locked-in syndrome. We identify a transitional phase of\n                      <jats:italic>communicative ambiguity<\/jats:italic>\n                      , in which residual ocular signals persist but can no longer be reliably attributed to intentional, patient-controlled communication. This phase is characterized by increasing inconsistency of signals and a divergence between observable responses and their interpretive certainty, creating uncertainty in assessing patient preferences.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Implications<\/jats:title>\n                    <jats:p>Communicative ambiguity represents a clinically underrecognized but critical threshold in advanced ALS, marking the transition from direct patient autonomy to interpretative and surrogate-based decision-making. Failure to recognize this phase risks misinterpretation of patient intent and may undermine goal-concordant care. Timely and iterative ACP, initiated before communication becomes unreliable, is essential. We further propose four clinical pathways for treatment goal conversations, highlighting their differing implications for timing, symptom burden, and ethical decision-making.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s42466-026-00522-3","type":"journal-article","created":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T15:30:12Z","timestamp":1785425412000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Ethical challenges in treatment-goal transitions in invasively ventilated ALS: a case-based topical review"],"prefix":"10.1186","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3258-543X","authenticated-orcid":false,"given":"Sarah K.","family":"Bublitz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedikt","family":"Becker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonia F.","family":"Demleitner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petra","family":"Dietz-Laukemann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Lingor","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benno","family":"Littger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Lorenzl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Berend","family":"Feddersen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,7,30]]},"reference":[{"key":"522_CR1","doi-asserted-by":"publisher","first-page":"566858","DOI":"10.3389\/fnins.2020.566858","volume":"14","author":"P Rojas","year":"2020","unstructured":"Rojas, P., Ram\u00edrez, A. I., Fern\u00e1ndez-Albarral, J. A., et al. (2020). Amyotrophic Lateral Sclerosis: A Neurodegenerative Motor Neuron Disease With Ocular Involvement. Front Neurosci, 14, 566858. https:\/\/doi.org\/10.3389\/fnins.2020.566858","journal-title":"Front Neurosci"},{"key":"522_CR2","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1016\/j.jns.2011.10.016","volume":"314","author":"HE Moss","year":"2012","unstructured":"Moss, H. E., McCluskey, L., Elman, L., et al. (2012). Cross-sectional evaluation of clinical neuro-ophthalmic abnormalities in an amyotrophic lateral sclerosis population. Journal Of The Neurological Sciences, 314, 97\u2013101. https:\/\/doi.org\/10.1016\/j.jns.2011.10.016","journal-title":"Journal Of The Neurological Sciences"},{"key":"522_CR3","doi-asserted-by":"publisher","first-page":"413","DOI":"10.1136\/jnnp.2010.232637","volume":"82","author":"P Bede","year":"2011","unstructured":"Bede, P., Oliver, D., Stodart, J., et al. (2011). Palliative care in amyotrophic lateral sclerosis: a review of current international guidelines and initiatives. Journal Of Neurology, Neurosurgery And Psychiatry, 82, 413\u2013418. https:\/\/doi.org\/10.1136\/jnnp.2010.232637","journal-title":"Journal Of Neurology, Neurosurgery And Psychiatry"},{"key":"522_CR4","doi-asserted-by":"publisher","first-page":"285","DOI":"10.1136\/jnnp-2019-322213","volume":"91","author":"N Hayashi","year":"2020","unstructured":"Hayashi, N., Atsuta, N., Yokoi, D., et al. (2020). Prognosis of amyotrophic lateral sclerosis patients undergoing tracheostomy invasive ventilation therapy in Japan. Journal Of Neurology, Neurosurgery And Psychiatry, 91, 285\u2013290. https:\/\/doi.org\/10.1136\/jnnp-2019-322213","journal-title":"Journal Of Neurology, Neurosurgery And Psychiatry"},{"key":"522_CR5","doi-asserted-by":"publisher","first-page":"1160","DOI":"10.1111\/ene.14647","volume":"28","author":"S Spittel","year":"2021","unstructured":"Spittel, S., Maier, A., Kettemann, D., et al. (2021). Non-invasive and tracheostomy invasive ventilation in amyotrophic lateral sclerosis: Utilization and survival rates in a cohort study over 12 years in Germany. European Journal Of Neurology, 28, 1160\u20131171. https:\/\/doi.org\/10.1111\/ene.14647","journal-title":"European Journal Of Neurology"},{"key":"522_CR6","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1016\/j.jns.2012.08.011","volume":"323","author":"R Spataro","year":"2012","unstructured":"Spataro, R., Bono, V., Marchese, S., & La Bella, V. (2012). Tracheostomy mechanical ventilation in patients with amyotrophic lateral sclerosis: Clinical features and survival analysis. Journal Of The Neurological Sciences, 323, 66\u201370. https:\/\/doi.org\/10.1016\/j.jns.2012.08.011","journal-title":"Journal Of The Neurological Sciences"},{"key":"522_CR7","doi-asserted-by":"publisher","first-page":"2730","DOI":"10.1007\/s00415-018-9054-x","volume":"265","author":"M Magelssen","year":"2018","unstructured":"Magelssen, M., Holm\u00f8y, T., Horn, M. A., et al. (2018). Ethical challenges in tracheostomy-assisted ventilation in amyotrophic lateral sclerosis. Journal Of Neurology, 265, 2730\u20132736. https:\/\/doi.org\/10.1007\/s00415-018-9054-x","journal-title":"Journal Of Neurology"},{"key":"522_CR8","doi-asserted-by":"publisher","first-page":"444","DOI":"10.1080\/21678421.2025.2495014","volume":"26","author":"N Turner","year":"2025","unstructured":"Turner, N., Palmer, J., Faull, C., et al. (2025). Tracheostomy ventilation in ALS: healthcare practitioner perspectives on quality of life and implications for decision-making. Amyotroph Lateral Scler Front Degener, 26, 444\u2013451. https:\/\/doi.org\/10.1080\/21678421.2025.2495014","journal-title":"Amyotroph Lateral Scler Front Degener"},{"key":"522_CR9","doi-asserted-by":"publisher","first-page":"802","DOI":"10.1016\/j.jpainsymman.2011.10.012","volume":"43","author":"JT Berger","year":"2012","unstructured":"Berger, J. T. (2012). Preemptive Use of Palliative Sedation and Amyotrophic Lateral Sclerosis. J Pain Symptom Manage, 43, 802\u2013805. https:\/\/doi.org\/10.1016\/j.jpainsymman.2011.10.012","journal-title":"J Pain Symptom Manage"},{"key":"522_CR10","doi-asserted-by":"publisher","first-page":"205","DOI":"10.1089\/jpm.2011.0133","volume":"15","author":"PS Dreyer","year":"2012","unstructured":"Dreyer, P. S., Felding, M., Klitn\u00e6s, C. S., & Lorenzen, C. K. (2012). Withdrawal of Invasive Home Mechanical Ventilation in Patients with Advanced Amyotrophic Lateral Sclerosis: Ten Years of Danish Experience. Journal Of Palliative Medicine, 15, 205\u2013209. https:\/\/doi.org\/10.1089\/jpm.2011.0133","journal-title":"Journal Of Palliative Medicine"},{"key":"522_CR11","doi-asserted-by":"publisher","first-page":"383","DOI":"10.1136\/practneurol-2016-001574","volume":"17","author":"A Gleeson","year":"2017","unstructured":"Gleeson, A., & Johnson, F. (2017). Withdrawal of invasive ventilation in a patient with motor neurone disease and total locked-in syndrome. Practical Neurology, 17, 383\u2013386. https:\/\/doi.org\/10.1136\/practneurol-2016-001574","journal-title":"Practical Neurology"},{"key":"522_CR12","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1080\/21678421.2016.1214734","volume":"18","author":"D Kettemann","year":"2017","unstructured":"Kettemann, D., Funke, A., Maier, A., et al. (2017). Clinical characteristics and course of dying in patients with amyotrophic lateral sclerosis withdrawing from long-term ventilation. Amyotroph Lateral Scler Front Degener, 18, 53\u201359. https:\/\/doi.org\/10.1080\/21678421.2016.1214734","journal-title":"Amyotroph Lateral Scler Front Degener"},{"key":"522_CR13","doi-asserted-by":"publisher","first-page":"325","DOI":"10.1007\/s00415-023-11957-y","volume":"271","author":"E Aust","year":"2024","unstructured":"Aust, E., Graupner, S-T., G\u00fcnther, R., et al. (2024). Impairment of oculomotor functions in patients with early to advanced amyotrophic lateral sclerosis. Journal Of Neurology, 271, 325\u2013339. https:\/\/doi.org\/10.1007\/s00415-023-11957-y","journal-title":"Journal Of Neurology"},{"key":"522_CR14","doi-asserted-by":"publisher","first-page":"857","DOI":"10.1001\/archneurol.2011.130","volume":"68","author":"R Sharma","year":"2011","unstructured":"Sharma, R. (2011). Oculomotor Dysfunction in Amyotrophic Lateral Sclerosis: A Comprehensive Review. Archives Of Neurology, 68, 857. https:\/\/doi.org\/10.1001\/archneurol.2011.130","journal-title":"Archives Of Neurology"},{"key":"522_CR15","doi-asserted-by":"publisher","first-page":"e0142546","DOI":"10.1371\/journal.pone.0142546","volume":"10","author":"M Gorges","year":"2015","unstructured":"Gorges, M., M\u00fcller, H-P., Lul\u00e9, D., et al. (2015). Eye Movement Deficits Are Consistent with a Staging Model of pTDP-43 Pathology in Amyotrophic Lateral Sclerosis. PLOS ONE, 10, e0142546. https:\/\/doi.org\/10.1371\/journal.pone.0142546","journal-title":"PLOS ONE"},{"key":"522_CR16","doi-asserted-by":"publisher","first-page":"107","DOI":"10.1186\/s40478-016-0379-3","volume":"4","author":"K Hayashi","year":"2016","unstructured":"Hayashi, K., Mochizuki, Y., Takeuchi, R., et al. (2016). Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked-in state (communication Stage V). Acta Neuropathol Commun, 4, 107. https:\/\/doi.org\/10.1186\/s40478-016-0379-3","journal-title":"Acta Neuropathol Commun"},{"key":"522_CR17","doi-asserted-by":"publisher","unstructured":"Poletti, B., Solca, F., Carelli, L., et al. (2021). Association of Clinically Evident Eye Movement Abnormalities With Motor and Cognitive Features in Patients With Motor Neuron Disorders. Neurology, 97. https:\/\/doi.org\/10.1212\/WNL.0000000000012774","DOI":"10.1212\/WNL.0000000000012774"},{"key":"522_CR18","doi-asserted-by":"publisher","first-page":"1399923","DOI":"10.3389\/fnins.2024.1399923","volume":"18","author":"MR Readman","year":"2024","unstructured":"Readman, M. R., Polden, M., Gibbs, M. C., et al. (2024). Oculomotor atypicalities in motor neurone disease: a systematic review. Front Neurosci, 18, 1399923. https:\/\/doi.org\/10.3389\/fnins.2024.1399923","journal-title":"Front Neurosci"},{"key":"522_CR19","doi-asserted-by":"publisher","first-page":"478","DOI":"10.1080\/21678421.2025.2501690","volume":"26","author":"D Shen","year":"2025","unstructured":"Shen, D., Liu, A., Yang, X., et al. (2025). Exploring oculomotor challenges in amyotrophic lateral sclerosis: a comprehensive review. Amyotroph Lateral Scler Front Degener, 26, 478\u2013484. https:\/\/doi.org\/10.1080\/21678421.2025.2501690","journal-title":"Amyotroph Lateral Scler Front Degener"},{"key":"522_CR20","doi-asserted-by":"publisher","first-page":"38","DOI":"10.3109\/21678421.2015.1055276","volume":"17","author":"Y Nakayama","year":"2016","unstructured":"Nakayama, Y., Shimizu, T., Mochizuki, Y., et al. (2016). Predictors of impaired communication in amyotrophic lateral sclerosis patients with tracheostomy-invasive ventilation. Amyotroph Lateral Scler Front Degener, 17, 38\u201346. https:\/\/doi.org\/10.3109\/21678421.2015.1055276","journal-title":"Amyotroph Lateral Scler Front Degener"},{"key":"522_CR21","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1080\/01658107.2022.2038638","volume":"46","author":"A Rekik","year":"2022","unstructured":"Rekik, A., Mrabet, S., Kacem, I., et al. (2022). Eye Movement Abnormalities in Amyotrophic Lateral Sclerosis in a Tunisian Cohort. Neuro-Ophthalmol, 46, 227\u2013235. https:\/\/doi.org\/10.1080\/01658107.2022.2038638","journal-title":"Neuro-Ophthalmol"},{"key":"522_CR22","doi-asserted-by":"publisher","first-page":"1236","DOI":"10.1038\/s41467-022-28859-8","volume":"13","author":"U Chaudhary","year":"2022","unstructured":"Chaudhary, U., Vlachos, I., Zimmermann, J. B., et al. (2022). Spelling interface using intracortical signals in a completely locked-in patient enabled via auditory neurofeedback training. Nature Communications, 13, 1236. https:\/\/doi.org\/10.1038\/s41467-022-28859-8","journal-title":"Nature Communications"},{"key":"522_CR23","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1212\/01.WNL.0000069925.02052.1F","volume":"61","author":"H Hayashi","year":"2003","unstructured":"Hayashi, H., & Oppenheimer, E. A. (2003). ALS patients on TPPV: Totally locked-in state, neurologic findings and ethical implications. Neurology, 61, 135\u2013137. https:\/\/doi.org\/10.1212\/01.WNL.0000069925.02052.1F","journal-title":"Neurology"},{"key":"522_CR24","doi-asserted-by":"publisher","first-page":"925","DOI":"10.1016\/j.clinph.2010.08.019","volume":"122","author":"AR Murguialday","year":"2011","unstructured":"Murguialday, A. R., Hill, J., Bensch, M., et al. (2011). Transition from the locked in to the completely locked-in state: A physiological analysis. Clinical Neurophysiology, 122, 925\u2013933. https:\/\/doi.org\/10.1016\/j.clinph.2010.08.019","journal-title":"Clinical Neurophysiology"},{"key":"522_CR25","doi-asserted-by":"publisher","first-page":"2060","DOI":"10.1056\/NEJMoa1608085","volume":"375","author":"MJ Vansteensel","year":"2016","unstructured":"Vansteensel, M. J., Pels, E. G. M., Bleichner, M. G., et al. (2016). Fully Implanted Brain\u2013Computer Interface in a Locked-In Patient with ALS. New England Journal Of Medicine, 375, 2060\u20132066. https:\/\/doi.org\/10.1056\/NEJMoa1608085","journal-title":"New England Journal Of Medicine"},{"key":"522_CR26","doi-asserted-by":"publisher","first-page":"96","DOI":"10.3988\/jcn.2021.17.1.96","volume":"17","author":"F Cozza","year":"2021","unstructured":"Cozza, F., Lizio, A., Greco, L. C., et al. (2021). Ocular Involvement Occurs Frequently at All Stages of Amyotrophic Lateral Sclerosis: Preliminary Experience in a Large Italian Cohort. J Clin Neurol, 17, 96. https:\/\/doi.org\/10.3988\/jcn.2021.17.1.96","journal-title":"J Clin Neurol"},{"key":"522_CR27","doi-asserted-by":"publisher","first-page":"1824","DOI":"10.1007\/s00415-023-12104-3","volume":"271","author":"E Luib","year":"2024","unstructured":"Luib, E., Demleitner, A. F., Cordts, I., et al. (2024). Reduced tear fluid production in neurological diseases: a cohort study in 708 patients. Journal Of Neurology, 271, 1824\u20131836. https:\/\/doi.org\/10.1007\/s00415-023-12104-3","journal-title":"Journal Of Neurology"},{"key":"522_CR28","doi-asserted-by":"publisher","first-page":"649","DOI":"10.1097\/WCO.0000000000000862","volume":"33","author":"N Pender","year":"2020","unstructured":"Pender, N., Pinto-Grau, M., & Hardiman, O. (2020). Cognitive and behavioural impairment in amyotrophic lateral sclerosis. Current Opinion In Neurology, 33, 649\u2013654. https:\/\/doi.org\/10.1097\/WCO.0000000000000862","journal-title":"Current Opinion In Neurology"},{"key":"522_CR29","doi-asserted-by":"publisher","first-page":"1077","DOI":"10.1212\/WNL.59.7.1077","volume":"59","author":"C Lomen-Hoerth","year":"2002","unstructured":"Lomen-Hoerth, C., Anderson, T., & Miller, B. (2002). The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology, 59, 1077\u20131079. https:\/\/doi.org\/10.1212\/WNL.59.7.1077","journal-title":"Neurology"},{"key":"522_CR30","doi-asserted-by":"publisher","first-page":"1632054","DOI":"10.3389\/fnins.2025.1632054","volume":"19","author":"S Tavakolan Haghighi","year":"2025","unstructured":"Tavakolan Haghighi, S., Bertasini, C., Picari, R., & Paolone, G. (2025). Decision making in frontotemporal dementia: neuropsychological and neuroimaging assessments. Front Neurosci, 19, 1632054. https:\/\/doi.org\/10.3389\/fnins.2025.1632054","journal-title":"Front Neurosci"},{"key":"522_CR31","doi-asserted-by":"publisher","first-page":"987","DOI":"10.1164\/rccm.200409-1295OC","volume":"171","author":"E Azoulay","year":"2005","unstructured":"Azoulay, E., Pochard, F., Kentish-Barnes, N., et al. (2005). Risk of Post-traumatic Stress Symptoms in Family Members of Intensive Care Unit Patients. American Journal Of Respiratory And Critical Care Medicine, 171, 987\u2013994. https:\/\/doi.org\/10.1164\/rccm.200409-1295OC","journal-title":"American Journal Of Respiratory And Critical Care Medicine"},{"key":"522_CR32","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1177\/0193945914544176","volume":"38","author":"AB Petrinec","year":"2016","unstructured":"Petrinec, A. B., & Daly, B. J. (2016). Post-Traumatic Stress Symptoms in Post-ICU Family Members: Review and Methodological Challenges. Western Journal Of Nursing Research, 38, 57\u201378. https:\/\/doi.org\/10.1177\/0193945914544176","journal-title":"Western Journal Of Nursing Research"},{"key":"522_CR33","doi-asserted-by":"publisher","first-page":"821","DOI":"10.1016\/j.jpainsymman.2016.12.331","volume":"53","author":"RL Sudore","year":"2017","unstructured":"Sudore, R. L., Lum, H. D., You, J. J., et al. (2017). Defining Advance Care Planning for Adults: A Consensus Definition From a Multidisciplinary Delphi Panel. J Pain Symptom Manage, 53, 821\u2013832e1. https:\/\/doi.org\/10.1016\/j.jpainsymman.2016.12.331","journal-title":"J Pain Symptom Manage"},{"key":"522_CR34","doi-asserted-by":"publisher","first-page":"e543","DOI":"10.1016\/S1470-2045(17)30582-X","volume":"18","author":"JAC Rietjens","year":"2017","unstructured":"Rietjens, J. A. C., Sudore, R. L., Connolly, M., et al. (2017). Definition and recommendations for advance care planning: an international consensus supported by the European Association for Palliative Care. The Lancet Oncology, 18, e543\u2013e551. https:\/\/doi.org\/10.1016\/S1470-2045(17)30582-X","journal-title":"The Lancet Oncology"},{"key":"522_CR35","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1186\/s42466-026-00481-9","volume":"8","author":"K Linse","year":"2026","unstructured":"Linse, K., Lul\u00e9, D., Sch\u00f6berl, F., et al. (2026). Between guidelines and reality: expert neurologists\u2019 perspectives on structural resources for ALS care in Germany. Neurol Res Pract, 8, 22. https:\/\/doi.org\/10.1186\/s42466-026-00481-9","journal-title":"Neurol Res Pract"},{"key":"522_CR36","doi-asserted-by":"publisher","DOI":"10.1002\/mus.28218","author":"SK Bublitz","year":"2024","unstructured":"Bublitz, S. K., Eham, M., Ellrott, H., et al. (2024). Homecare amyotrophic lateral sclerosis (ALS): A multidisciplinary, home-based model of care for patients with ALS and their caregivers. Muscle And Nerve. https:\/\/doi.org\/10.1002\/mus.28218","journal-title":"Muscle And Nerve"},{"key":"522_CR37","doi-asserted-by":"publisher","first-page":"e16264","DOI":"10.1111\/ene.16264","volume":"31","author":"P Van Damme","year":"2024","unstructured":"Van Damme, P., Al-Chalabi, A., Andersen, P. M., et al. (2024). European Academy of Neurology (EAN) guideline on the management of amyotrophic lateral sclerosis in collaboration with European Reference Network for Neuromuscular Diseases (ERN EURO\u2010NMD). European Journal Of Neurology, 31, e16264. https:\/\/doi.org\/10.1111\/ene.16264","journal-title":"European Journal Of Neurology"},{"key":"522_CR38","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1186\/s42466-023-00251-x","volume":"5","author":"S Petri","year":"2023","unstructured":"Petri, S., Grehl, T., Grosskreutz, J., et al. (2023). Guideline Motor neuron diseases of the German Society of Neurology (Deutsche Gesellschaft f\u00fcr Neurologie). Neurol Res Pract, 5, 25. https:\/\/doi.org\/10.1186\/s42466-023-00251-x","journal-title":"Neurol Res Pract"},{"key":"522_CR39","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1177\/02692163251396020","volume":"40","author":"WKV Chung","year":"2026","unstructured":"Chung, W. K. V., Chan, K. P., Hsu, D. Y., et al. (2026). A 10-year service evaluation of a multidisciplinary neuro-palliative care model in motor neuron disease: Impact on palliative care service delivery & advance care planning. Palliative Medicine, 40, 253\u2013264. https:\/\/doi.org\/10.1177\/02692163251396020","journal-title":"Palliative Medicine"},{"key":"522_CR40","doi-asserted-by":"publisher","first-page":"191","DOI":"10.1186\/s12904-024-01524-1","volume":"23","author":"AL Mangal","year":"2024","unstructured":"Mangal, A. L., M\u00fccke, M., Rolke, R., & Appelmann, I. (2024). Advance directives in amyotrophic lateral sclerosis \u2013 a systematic review and meta-analysis. BMC Palliat Care, 23, 191. https:\/\/doi.org\/10.1186\/s12904-024-01524-1","journal-title":"BMC Palliat Care"},{"key":"522_CR41","doi-asserted-by":"publisher","first-page":"1131","DOI":"10.1007\/s00115-024-01769-4","volume":"95","author":"C Eickhoff","year":"2024","unstructured":"Eickhoff, C., Sch\u00f6ne-Seifert, B., Kettemann, D., et al. (2024). Perspektiven auf das Lebensende: eine systematische Erhebung bei Patienten mit amyotropher Lateralsklerose. Der Nervenarzt, 95, 1131\u20131138. https:\/\/doi.org\/10.1007\/s00115-024-01769-4","journal-title":"Der Nervenarzt"},{"key":"522_CR42","doi-asserted-by":"publisher","first-page":"572","DOI":"10.1177\/02692163241242320","volume":"38","author":"I Vandenbogaerde","year":"2024","unstructured":"Vandenbogaerde, I., Van Den Block, L., Deliens, L., et al. (2024). Experiences with advance care planning in amyotrophic lateral sclerosis: Qualitative longitudinal study with people with amyotrophic lateral sclerosis and their family carers. Palliative Medicine, 38, 572\u2013581. https:\/\/doi.org\/10.1177\/02692163241242320","journal-title":"Palliative Medicine"},{"key":"522_CR43","doi-asserted-by":"publisher","first-page":"e0290508","DOI":"10.1371\/journal.pone.0290508","volume":"18","author":"RM Van Eenennaam","year":"2023","unstructured":"Van Eenennaam, R. M., Rave, N., Kruithof, W. J., et al. (2023). Control in the absence of choice: A qualitative study on decision-making about gastrostomy in people with amyotrophic lateral sclerosis, caregivers, and healthcare professionals. PLOS ONE, 18, e0290508. https:\/\/doi.org\/10.1371\/journal.pone.0290508","journal-title":"PLOS ONE"},{"key":"522_CR44","doi-asserted-by":"publisher","first-page":"977","DOI":"10.1016\/S0277-9536(98)00411-0","volume":"48","author":"GL Albrecht","year":"1999","unstructured":"Albrecht, G. L., & Devlieger, P. J. (1999). The disability paradox: high quality of life against all odds. Social Science And Medicine, 48, 977\u2013988. https:\/\/doi.org\/10.1016\/S0277-9536(98)00411-0","journal-title":"Social Science And Medicine"},{"key":"522_CR45","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1186\/s12904-024-01443-1","volume":"23","author":"E Wilson","year":"2024","unstructured":"Wilson, E., Palmer, J., Armstrong, A., et al. (2024). End of life decision making when home mechanical ventilation is used to sustain breathing in Motor Neurone Disease: patient and family perspectives. BMC Palliat Care, 23, 115. https:\/\/doi.org\/10.1186\/s12904-024-01443-1","journal-title":"BMC Palliat Care"},{"key":"522_CR46","doi-asserted-by":"publisher","first-page":"821","DOI":"10.3390\/brainsci14080821","volume":"14","author":"N Turner","year":"2024","unstructured":"Turner, N., Faull, C., Palmer, J., et al. (2024). Understanding Quality of Life for People with Motor Neurone Disease Who Use Tracheostomy Ventilation and Family Members: A Scoping Review. Brain Sci, 14, 821. https:\/\/doi.org\/10.3390\/brainsci14080821","journal-title":"Brain Sci"},{"key":"522_CR47","doi-asserted-by":"publisher","first-page":"368","DOI":"10.1016\/S1474-4422(13)70026-7","volume":"12","author":"LH Goldstein","year":"2013","unstructured":"Goldstein, L. H., & Abrahams, S. (2013). Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment. Lancet Neurology, 12, 368\u2013380. https:\/\/doi.org\/10.1016\/S1474-4422(13)70026-7","journal-title":"Lancet Neurology"},{"key":"522_CR48","doi-asserted-by":"publisher","first-page":"600","DOI":"10.3390\/brainsci15060600","volume":"15","author":"G Artioli","year":"2025","unstructured":"Artioli, G., Guardamagna, L., Succi, N., et al. (2025). Relational, Ethical, and Care Challenges in ALS: A Systematic Review and Qualitative Metasynthesis of Nurses\u2019 Perspectives. Brain Sci, 15, 600. https:\/\/doi.org\/10.3390\/brainsci15060600","journal-title":"Brain Sci"},{"key":"522_CR49","doi-asserted-by":"publisher","first-page":"221","DOI":"10.1177\/02692163241291745","volume":"39","author":"M Walls","year":"2025","unstructured":"Walls, M., Claffey, A., Mockler, D., & Galvin, M. (2025). Working with people living with motor neurone disease and the impact on professionals\u2019 emotional and psychological well-being: A scoping review. Palliative Medicine, 39, 221\u2013244. https:\/\/doi.org\/10.1177\/02692163241291745","journal-title":"Palliative Medicine"},{"key":"522_CR50","doi-asserted-by":"publisher","unstructured":"Chan, Y., Creer, S., Mere, J., et al. (2026). The Emotional Experiences of Healthcare Professionals Working in Amyotrophic Lateral Sclerosis: A Systematic Review. Muscle Nerve mus, 70309. https:\/\/doi.org\/10.1002\/mus.70309","DOI":"10.1002\/mus.70309"},{"key":"522_CR51","doi-asserted-by":"publisher","first-page":"263235242311708","DOI":"10.1177\/26323524231170881","volume":"17","author":"S Daneau","year":"2023","unstructured":"Daneau, S., Bourbonnais, A., Allard, \u00c9., et al. (2023). Intensive palliative care\u2019: a qualitative study of issues related to nurses\u2019 care of people with amyotrophic lateral sclerosis at end-of-life. Palliat Care Soc Pract, 17, 26323524231170881. https:\/\/doi.org\/10.1177\/26323524231170881","journal-title":"Palliat Care Soc Pract"},{"key":"522_CR52","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1186\/s42466-024-00328-1","volume":"6","author":"K Linse","year":"2024","unstructured":"Linse, K., Weber, C., Reilich, P., et al. (2024). Patients\u2019 and caregivers\u2019 perception of multidimensional and palliative care in amyotrophic lateral sclerosis \u2013 protocol of a German multicentre study. Neurol Res Pract, 6, 34. https:\/\/doi.org\/10.1186\/s42466-024-00328-1","journal-title":"Neurol Res Pract"},{"key":"522_CR53","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1136\/bmjspcare-2014-000826","volume":"7","author":"K Phelps","year":"2017","unstructured":"Phelps, K., Regen, E., Oliver, D., et al. (2017). Withdrawal of ventilation at the patient\u2019s request in MND: a retrospective exploration of the ethical and legal issues that have arisen for doctors in the UK. BMJ Support Palliat Care, 7, 189\u2013196. https:\/\/doi.org\/10.1136\/bmjspcare-2014-000826","journal-title":"BMJ Support Palliat Care"}],"container-title":["Neurological Research and Practice"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s42466-026-00522-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s42466-026-00522-3","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s42466-026-00522-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T05:03:54Z","timestamp":1785474234000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1186\/s42466-026-00522-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7,30]]},"references-count":53,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["522"],"URL":"https:\/\/doi.org\/10.1186\/s42466-026-00522-3","relation":{},"ISSN":["2524-3489"],"issn-type":[{"value":"2524-3489","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7,30]]},"assertion":[{"value":"21 May 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"23 July 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 July 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Ethics approval was obtained from the ethics commission of the Ludwig-Maximilians-University of Munich (project no 21-0991).","order":1,"name":"Ethics","label":"Ethics approval","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All patients or their legal representatives provided informed consent for participation in the project Homecare ALS\/KOmpASS ALS.","order":2,"name":"Ethics","label":"Consent to participate","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All patients or their legal representatives provided written informed consent for participation in the accompanying Homecare ALS\/KOmpASS ALS study, including the pseudonymized evaluation of clinical and healthcare-related data.","order":3,"name":"Ethics","label":"Consent for publication","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","label":"Competing interests","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"59"}}