REVEALS-a longitudinal cohort study of multifaceted respiratory assessment in ALS.

Autor:	Rooney J; Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland.; Institute and Clinic for Occupational, Social and Environmental Medicine, University Hospital, LMU Munich, Munich, Germany., Murray D; Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland.; Beaumont Hospital, Dublin, Ireland., Meldrum D; Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland., Al-Chalabi A; Department of Basic and Clinical Neuroscience, King's College London, Maurice Wohl Clinical Neuroscience Institute, London, UK.; Department of Neurology, King's College Hospital, London, UK., Bunte T; UMC Utrecht, Utrecht, The Netherlands., Chiwera T; Department of Basic and Clinical Neuroscience, King's College London, Maurice Wohl Clinical Neuroscience Institute, London, UK.; Department of Neurology, King's College Hospital, London, UK., Choudhury M; Department of Basic and Clinical Neuroscience, King's College London, Maurice Wohl Clinical Neuroscience Institute, London, UK.; Department of Neurology, King's College Hospital, London, UK., Chio A; ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.; Neurology 1, Azienda Ospedale Università Città della Salute e della Scienza, Turin, Italy., Fenton L; Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland., Fortune J; Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland., Maidment L; Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK., Manera U; ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.; Neurology 1, Azienda Ospedale Università Città della Salute e della Scienza, Turin, Italy., McDermott CJ; Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.; Department of Neuroscience, Sheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, UK., Meyjes M; UMC Utrecht, Utrecht, The Netherlands., Tattersall R; Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland.; Beaumont Hospital, Dublin, Ireland., Torrieri MC; ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy., Van Damme P; Neurology Department, KU Leuven, University Hospitals Leuven, Leuven, Belgium, and.; Department of Neuroscience, KU Leuven, Leuven Brain Institute and VIB Center for Brain & Disease Research, Leuven, Belgium., Vanderlinden E; Neurology Department, KU Leuven, University Hospitals Leuven, Leuven, Belgium, and., Wood C; Department of Basic and Clinical Neuroscience, King's College London, Maurice Wohl Clinical Neuroscience Institute, London, UK.; Department of Neurology, King's College Hospital, London, UK., van den Berg LH; UMC Utrecht, Utrecht, The Netherlands., Hardiman O; Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland.; Beaumont Hospital, Dublin, Ireland.
Jazyk:	angličtina
Zdroj:	Amyotrophic lateral sclerosis & frontotemporal degeneration [Amyotroph Lateral Scler Frontotemporal Degener] 2024 Nov; Vol. 25 (7-8), pp. 661-671. Date of Electronic Publication: 2024 Jun 06.
DOI:	10.1080/21678421.2024.2359556
Abstrakt:	Objective: To systematically assess decline in respiratory measures in amyotrophic lateral sclerosis (ALS) and to examine the impact of sex, disease onset type and baseline morbidity on progression. Methods: The REVEALS study (Registry of Endpoints and Validated Experiences in ALS) was conducted between April 2018 and February 2021 in six European ALS centers. Slow and forced vital capacity (S/FVC), sniff nasal inspiratory pressure (SNIP), peak cough flow, amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R), and respiratory morbidity were collected. Data were analyzed using a Bayesian multiple outcomes random effects model. Results: Two hundred and eighty participants had a median of three assessments (IQR 2.0, 5.0) over a median of 8 months (IQR 2.3, 14.1). There were 974 data collection timepoints. Differences in respiratory measures and rates of decline between disease-onset and sex subgroups were identified. Females had lower scores in all respiratory measures and females with bulbar onset ALS had faster decline compared with other sub-groups. These differences were not detected by the ALSFRS-r respiratory subscale. Dyspnea, orthopnea, and a higher King's stage at baseline were associated with lower respiratory scores throughout follow-up, while having a regular productive cough at baseline was associated with lower peak cough flow scores. Conclusion: Respiratory function declines more quickly in females with ALS compared with males when measured by FVC, SVC, SNIP, or PCF, but not the ALSFRS-R respiratory sub-score. Higher baseline King's staging and the presence of clinical respiratory symptoms at baseline were associated with worse respiratory function. The ALSFRS-R respiratory sub-score is poorly correlated with objective respiratory measurements.
Databáze:	MEDLINE
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