{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T14:19:17Z","timestamp":1787581157148,"version":"build-2736575974"},"reference-count":36,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2024,3,20]],"date-time":"2024-03-20T00:00:00Z","timestamp":1710892800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2024,3,20]],"date-time":"2024-03-20T00:00:00Z","timestamp":1710892800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neuromuscular Diseases"],"published-print":{"date-parts":[[2024,4,30]]},"abstract":"<jats:sec specific-use=\"heading-level-1\">\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Hereditary proximal spinal muscular atrophy (SMA) is characterized by abnormal alpha motor neuron function in brainstem and spinal cord. Bulbar dysfunction, including limited mouth opening, is present in the majority of patients with SMA but it is unknown if and how these problems change during disease course.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec specific-use=\"heading-level-1\">\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>In this prospective, observational, longitudinal natural history study we aimed to study bulbar dysfunction in patients with SMA types 2 and 3.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec specific-use=\"heading-level-1\">\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>\n                      We included 44 patients with SMA types 2 and 3 (mean age was 33.6 (95% CI 28.4;38.9) and re-examined them after on average 4 years. None were treated with\n                      <jats:italic toggle=\"yes\">SMN-<\/jats:italic>\n                      modulating treatments before or during the course of this study. Longitudinal assessments included a questionnaire on mandibular and bulbar function, the Mandibular Function Impairment Questionnaire (MFIQ), and a clinical examination of masticatory performance, maximum voluntary bite force, and mandibular movements including the active maximal mouth opening.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec specific-use=\"heading-level-1\">\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      We found significant higher MFIQ scores and a significant decrease of all mandibular movements in patients with SMA type 2 (\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u200a&lt;\u200a0.001), but not in SMA type 3. Masticatory performance and maximum voluntary bite force did not change significantly. Mean reduction of active maximal mouth opening at follow-up was 3.5\u200amm in SMA type 2 (95% CI: 2.3; 4.7,\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u200a&lt;\u200a0.001). SMA type 2 was an independent predictor for a more severe reduction of the mouth opening (\u03b2=\u200a\u20132.0\u200amm (95% CI: \u20133.8; \u20130.1,\n                      <jats:italic toggle=\"yes\">p<\/jats:italic>\n                      \u200a=\u200a0.043)).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec specific-use=\"heading-level-1\">\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>Bulbar functions such as mandibular mobility and active maximum mouth opening decrease significantly over the course of four years in patients with SMA type 2.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3233\/jnd-240007","type":"journal-article","created":{"date-parts":[[2024,3,22]],"date-time":"2024-03-22T12:04:38Z","timestamp":1711109078000},"page":"655-664","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":8,"title":["Natural History of Mandibular Function in Spinal Muscular Atrophy Types 2 and 3"],"prefix":"10.1177","volume":"11","author":[{"given":"H. Willemijn","family":"van Bruggen","sequence":"first","affiliation":[{"name":"Radboud University Nijmegen Medical Center","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camiel A.","family":"Wijngaarde","sequence":"additional","affiliation":[{"name":"University Medical Center Utrecht","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faylynn","family":"Asselman","sequence":"additional","affiliation":[{"name":"University Medical Center Utrecht","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marloes","family":"Stam","sequence":"additional","affiliation":[{"name":"University Medical Center Utrecht","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nico H.J.","family":"Creugers","sequence":"additional","affiliation":[{"name":"Radboud University Nijmegen Medical Center","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renske I.","family":"Wadman","sequence":"additional","affiliation":[{"name":"University Medical Center Utrecht","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W. Ludo","family":"van der Pol","sequence":"additional","affiliation":[{"name":"University Medical Center Utrecht","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stanimira I.","family":"Kalaykova","sequence":"additional","affiliation":[{"name":"Radboud University Nijmegen Medical Center","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2024,3,20]]},"reference":[{"key":"e_1_3_4_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90460-3"},{"key":"e_1_3_4_3_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1961.00450140022003"},{"key":"e_1_3_4_4_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181c34aa6"},{"key":"e_1_3_4_5_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000000796"},{"key":"e_1_3_4_6_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000002348"},{"key":"e_1_3_4_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2008.02.008"},{"key":"e_1_3_4_8_2","doi-asserted-by":"publisher","DOI":"10.1177\/0883073811407696"},{"key":"e_1_3_4_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2011.08.016"},{"key":"e_1_3_4_10_2","doi-asserted-by":"publisher","DOI":"10.1097\/EJA.0000000000000838"},{"key":"e_1_3_4_11_2","doi-asserted-by":"publisher","DOI":"10.3233\/JND-190379"},{"key":"e_1_3_4_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0889-5406(99)70296-9"},{"key":"e_1_3_4_13_2","doi-asserted-by":"crossref","unstructured":"WadmanRI StamM GijzenM LemminkHH SnoeckIN WijngaardeCA et alAssociation of motor milestones SMN2 copy and outcome in spinal muscular atrophy types 0\u20134. 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