{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T09:31:20Z","timestamp":1793266280830,"version":"4.5.0"},"reference-count":24,"publisher":"SAGE Publications","issue":"6","license":[{"start":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T00:00:00Z","timestamp":1773360000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T00:00:00Z","timestamp":1773360000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"DOI":"10.13039\/501100002661","name":"Fonds De La Recherche Scientifique - FNRS","doi-asserted-by":"publisher","award":["J.0047.20"],"award-info":[{"award-number":["J.0047.20"]}],"id":[{"id":"10.13039\/501100002661","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neuromuscular Diseases"],"published-print":{"date-parts":[[2026,11]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Spinal muscular atrophy (SMA) is a genetic neuromuscular disease characterized by muscular atrophy and respiratory and bulbar dysfunction, especially in the most severe phenotypes (SMA type 0 and 1). Swallowing function remains understudied because assessment tools are often unavailable or poorly tolerated by young children. The Iowa Oral Performance Instrument (IOPI), that measures orofacial strength via a small air-filled bulb has recently been evaluated in SMA type 2 and 3 patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>\n                      The purpose of this study was to investigate muscle strength of several muscles used during the oral phase of swallowing using IOPI in SMA type 1 children treated with nusinersen compared to healthy controls. In addition, it sought to correlate labial and lingual pressures with known key predictors of phenotype severity (Children's Hospital Of Philadelphia Infant Test of Neuromuscular Disorders [CHOP INTEND], age at first symptoms,\n                      <jats:italic toggle=\"yes\">SMN2<\/jats:italic>\n                      copy number) and indicators of feeding-nutritional status (p-FOIS (Pediatric Functional Oral Intake Scale)).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>Combining results from four independent centers, we recruited 22 individuals with a confirmed genetic diagnosis of symptomatic SMA type 1. All patients had been treated with nusinersen for at least 2.5 years and had undergone at least one IOPI measurement at a median age of 5.4 years. IOPI data were compared to age- and gender-matched controls and published normative data when available.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Oral pressures were lower in patients than in typically developing children (lips:\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u2009&lt;\u20090.01; tongue:\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u2009&lt;\u20090.001). Weaker labial pressure was significantly correlated with younger age at disease onset (r\u2009=\u20090.66;\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u2009=\u20090.0053), older age at first nusinersen injection (r\u2009=\u2009-0.52;\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u2009=\u20090.0326), lower body mass index (r\u2009=\u20090.61;\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u2009=\u20090.0149), and worse motor performance as expressed by the CHOP INTEND score (r\u2009=\u20090.60;\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u2009=\u20090.0170). Weaker tongue pressure was significantly correlated with younger age at disease onset (r\u2009=\u20090.49;\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u2009=\u20090.0372) and lower p-FOIS (r\u2009=\u20090.526;\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u2009=\u20090.022).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>IOPI measurements objectively demonstrate orofacial weakness in this population compared with healthy controls and may serve as a biomarker to monitor the impact of comprehensive management, including disease-modifying therapies, on oral swallowing function in SMA type 1.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/22143602261433495","type":"journal-article","created":{"date-parts":[[2026,3,14]],"date-time":"2026-03-14T05:47:26Z","timestamp":1773467246000},"page":"1242-1251","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Impairment of lip and tongue strength in symptomatic SMA1 patients: Results from a 4-center prospective study using the IOPI"],"prefix":"10.1177","volume":"13","author":[{"given":"Charlotte","family":"Colot","sequence":"first","affiliation":[{"name":"Department of Paediatric Neurology and Neuromuscular Reference Center, H\u00f4pital Universitaire des Enfants Reine Fabiola (HUDERF)- H\u00f4pital Universitaire de Bruxelles (HUB)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Trucco","sequence":"additional","affiliation":[{"name":"University of Genova; Paediatric Neurology and Muscular Diseases Unit, IRCCS Istituto Giannina Gaslini"},{"name":"Neurorehabilitation Unit, The NeMo Clinical Center in Milan, University of Milan, Milan, Italy"},{"name":"Centro Clinico Nemo, Fondazione Serena Onlus, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2397-1240","authenticated-orcid":false,"given":"Pablo","family":"Ruiz Chicaiza","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurology and Neuromuscular Reference Center, H\u00f4pital Universitaire des Enfants Reine Fabiola (HUDERF)- H\u00f4pital Universitaire de Bruxelles (HUB)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elke","family":"Everaert","sequence":"additional","affiliation":[{"name":"Neuromuscular Reference Center (NMRC) Ghent University Hospital (UZ Gent)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Muys","sequence":"additional","affiliation":[{"name":"Neuromuscular Reference Center (NMRC) Ghent University Hospital (UZ Gent)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentine","family":"Tahon","sequence":"additional","affiliation":[{"name":"Neuromuscular Reference Center (NMRC) Ghent University Hospital (UZ Gent)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linde","family":"Van Himme","sequence":"additional","affiliation":[{"name":"Neuromuscular Reference Center (NMRC) Ghent University Hospital (UZ Gent)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elke","family":"Devos","sequence":"additional","affiliation":[{"name":"Neuromuscular Reference Center (NMRC) Ghent University Hospital (UZ Gent)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnaud","family":"Vanlander","sequence":"additional","affiliation":[{"name":"Neuromuscular Reference Center (NMRC) Ghent University Hospital (UZ Gent)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Lizio","sequence":"additional","affiliation":[{"name":"Neurorehabilitation Unit, The NeMo Clinical Center in Milan, University of Milan, Milan, Italy"},{"name":"Centro Clinico Nemo, Fondazione Serena Onlus, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Salmin","sequence":"additional","affiliation":[{"name":"Neurorehabilitation Unit, The NeMo Clinical Center in Milan, University of Milan, Milan, Italy"},{"name":"Centro Clinico Nemo, Fondazione Serena Onlus, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giorgia","family":"Coratti","sequence":"additional","affiliation":[{"name":"Universit\u00e0 Cattolica del Sacro Cuore"},{"name":"Centro Clinico Nemo, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emilio","family":"Albamonte","sequence":"additional","affiliation":[{"name":"Neurorehabilitation Unit, The NeMo Clinical Center in Milan, University of Milan, Milan, Italy"},{"name":"Centro Clinico Nemo, Fondazione Serena Onlus, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Stanca","sequence":"additional","affiliation":[{"name":"Universit\u00e0 Cattolica del Sacro Cuore"},{"name":"Centro Clinico Nemo, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marika","family":"Pane","sequence":"additional","affiliation":[{"name":"Universit\u00e0 Cattolica del Sacro Cuore"},{"name":"Centro Clinico Nemo, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eugenio","family":"Mercuri","sequence":"additional","affiliation":[{"name":"Universit\u00e0 Cattolica del Sacro Cuore"},{"name":"Centro Clinico Nemo, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Sansone","sequence":"additional","affiliation":[{"name":"University of Genova; 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Onasemnogene abeparvovec (AVXS-101) for the treatment of spinal muscular atrophy. J Pediatr Pharmacol Ther 2021 Jul 1; 26: 437\u2013444.","journal-title":"J Pediatr Pharmacol Ther"},{"key":"e_1_3_4_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.25101"},{"key":"e_1_3_4_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2017.11.005"},{"key":"e_1_3_4_8_2","doi-asserted-by":"publisher","DOI":"10.3233\/JND-240017"},{"key":"e_1_3_4_9_2","doi-asserted-by":"publisher","DOI":"10.3233\/JND-200614"},{"key":"e_1_3_4_10_2","doi-asserted-by":"crossref","unstructured":"Trucco F Salmin F Lizio A et al. Assessing Prevalence and Characteristics of Oro-bulbar Involvement in Children and Adults with SMA Type 2 and 3 Using a Multimodal Approach. Dysphagia [Internet]. 2023 Jun 8 [cited 2023 Sep 11]; Available from: https:\/\/link.springer.com\/10.1007\/s00455-023-10584-z.","DOI":"10.1007\/s00455-023-10584-z"},{"key":"e_1_3_4_11_2","doi-asserted-by":"publisher","DOI":"10.3109\/09638288.2014.921245"},{"key":"e_1_3_4_12_2","article-title":"Longitudinal assessment of strength, functional capacity, oropharyngeal function, and quality of life in oculopharyngeal muscular dystrophy","volume":"97","author":"Kroon R","unstructured":"Kroon R, Kalf J, De Swart B, et al. Longitudinal assessment of strength, functional capacity, oropharyngeal function, and quality of life in oculopharyngeal muscular dystrophy. Neurology. 2021 Oct 12; 97(15).","journal-title":"Neurology"},{"key":"e_1_3_4_13_2","article-title":"Association between maximum tongue pressure and swallowing safety and efficacy in amyotrophic lateral sclerosis","volume":"32","author":"Pizzorni N","unstructured":"Pizzorni N, Ginocchio D, Bianchi F, et al. Association between maximum tongue pressure and swallowing safety and efficacy in amyotrophic lateral sclerosis. Neurogastroenterol Motil 2020 Aug; 32: e13859.","journal-title":"Neurogastroenterol Motil"},{"key":"e_1_3_4_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.anl.2020.10.007"},{"key":"e_1_3_4_15_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00455-020-10116-z"},{"key":"e_1_3_4_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2009.11.014"},{"key":"e_1_3_4_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2020.12.007"},{"key":"e_1_3_4_18_2","doi-asserted-by":"publisher","DOI":"10.3233\/JND-190379"},{"key":"e_1_3_4_19_2","unstructured":"Prior TW Leach ME Finanger E. Spinal Muscular Atrophy. In: Adam MP Feldman J Mirzaa GM Pagon RA Wallace SE Bean LJ et al. editors. GeneReviews\u00ae [Internet]. Seattle (WA): University of Washington Seattle; 1993 [cited 2024 Jul 28]. Available from: http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1352\/."},{"key":"e_1_3_4_20_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.27809"},{"key":"e_1_3_4_21_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000000796"},{"key":"e_1_3_4_22_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000002348"},{"key":"e_1_3_4_23_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1702752"},{"key":"e_1_3_4_24_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1710504"},{"key":"e_1_3_4_25_2","first-page":"54","article-title":"Real-world respiratory and bulbar comorbidities of SMA type 1 children treated with nusinersen: 2-year single centre Australian experience","volume":"39","author":"Chen KA","unstructured":"Chen KA, Widger J, Teng A, et al. Real-world respiratory and bulbar comorbidities of SMA type 1 children treated with nusinersen: 2-year single centre Australian experience. Paediatr Respir Rev 2021 Sep; 39: 54\u201360.","journal-title":"Paediatr Respir Rev"}],"container-title":["Journal of Neuromuscular Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/22143602261433495","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/22143602261433495","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/22143602261433495","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T08:31:17Z","timestamp":1793262677000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/22143602261433495"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,13]]},"references-count":24,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2026,11]]}},"alternative-id":["10.1177\/22143602261433495"],"URL":"https:\/\/doi.org\/10.1177\/22143602261433495","relation":{},"ISSN":["2214-3599","2214-3602"],"issn-type":[{"value":"2214-3599","type":"print"},{"value":"2214-3602","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,13]]}}}