{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,9]],"date-time":"2026-05-09T07:11:05Z","timestamp":1778310665616,"version":"3.51.4"},"reference-count":12,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T00:00:00Z","timestamp":1777680000000},"content-version":"vor","delay-in-days":1,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100003500","name":"Universit\u00e0 degli Studi di Padova","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100003500","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neurol Sci"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Spinal and Bulbar Muscular Atrophy (SBMA) is a rare X-linked polyglutamine disorder characterized by a CAG trinucleotide repeat expansion in the androgen receptor gene. This leads to progressive lower motor neuron degeneration and skeletal muscle atrophy. Given the need for sensitive outcome measures in clinical trials, this study aimed to perform the linguistic adaptation and psychometric validation of the Adult Myopathy Assessment Tool (AMAT) for the Italian population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Following a rigorous forward-back translation protocol to ensure semantic and conceptual equivalence, the Italian AMAT was administered to 29 patients. The validation process assessed internal consistency (Cronbach\u2019s alpha), inter-rater and intra-rater reliability, and construct validity. The latter was evaluated through correlations with established clinical markers, including the Six-Minute Walk Test (6MWT), the SBMA Functional Rating Scale (SBMAFRS), and the ALSAQ-40 scale.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Psychometric analysis revealed excellent inter- and intra-rater reliability and strong internal consistency (Cronbach\u2019s alpha\u2009&gt;\u20090.70). Construct validity was confirmed through significant correlations with established functional markers, including the six-minute walk test (6MWT) and the SBMA Functional Rating Scale (SBMAFRS), while the expected negative correlations with ALSAQ-40 scale physical domains\u2014coupled with a lack of correlation with the communication domain\u2014affirmed divergent validity.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The Italian version of the AMAT is a reliable and valid instrument for quantifying functional impairment and endurance in SBMA. Its implementation facilitates standardized longitudinal assessment and enhances the feasibility of cross-national collaborative research.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s10072-026-09052-x","type":"journal-article","created":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T01:11:51Z","timestamp":1777684311000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Reliability and construct validity of the Italian version of AMAT scale in SBMA subjects"],"prefix":"10.1007","volume":"47","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4420-8076","authenticated-orcid":false,"given":"Elisabetta","family":"Pupillo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisa","family":"Bianchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maurizio A.","family":"Leone","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sabrina","family":"Paganoni","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alberto","family":"Romito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"Paredi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giacomo Maria","family":"Minicuci","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gianni","family":"Sorar\u00f9","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,5,2]]},"reference":[{"issue":"3","key":"9052_CR1","doi-asserted-by":"publisher","first-page":"317","DOI":"10.1007\/s12031-015-0674-7","volume":"58","author":"KH Fischbeck","year":"2016","unstructured":"Fischbeck KH (2016) Spinal and Bulbar Muscular Atrophy Overview. J Mol Neurosci 58(3):317\u2013320. https:\/\/doi.org\/10.1007\/s12031-015-0674-7","journal-title":"J Mol Neurosci"},{"issue":"1\u20132","key":"9052_CR2","doi-asserted-by":"publisher","first-page":"100","DOI":"10.1016\/j.jns.2007.08.012","volume":"264","author":"G Sorar\u00f9","year":"2008","unstructured":"Sorar\u00f9 G, D\u2019Ascenzo C, Polo A, Palmieri A, Baggio L, Vergani L, Gellera C, Moretto G, Pegoraro E, Angelini C (2008) Spinal and bulbar muscular atrophy: skeletal muscle pathology in male patients and heterozygous females. J Neurol Sci 264(1\u20132):100\u2013105.","journal-title":"J Neurol Sci"},{"issue":"6330","key":"9052_CR3","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1038\/352077a0","volume":"352","author":"AR La Spada","year":"1991","unstructured":"La Spada AR, Wilson EM, Lubahn DB, Harding AE, Fischbeck KH (1991) Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature 352(6330):77\u201379. https:\/\/doi.org\/10.1038\/352077a0","journal-title":"Nature"},{"issue":"3","key":"9052_CR4","doi-asserted-by":"publisher","first-page":"565","DOI":"10.1007\/s00415-018-8968-7","volume":"266","author":"M Breza","year":"2019","unstructured":"Breza M, Koutsis G (2019) Kennedy\u2019s disease (spinal and bulbar muscular atrophy): a clinically oriented review of a rare disease. J Neurol 266(3):565\u2013573. https:\/\/doi.org\/10.1007\/s00415-018-8968-7","journal-title":"J Neurol"},{"issue":"Pt 12","key":"9052_CR5","doi-asserted-by":"publisher","first-page":"3242","DOI":"10.1093\/brain\/awp258","volume":"132","author":"LE Rhodes","year":"2009","unstructured":"Rhodes LE, Freeman BK, Auh S, Kokkinis AD, La Pean A, Chen C, Lehky TJ, Shrader JA, Levy EW, Harris-Love M, Di Prospero NA, Fischbeck KH (2009) Clinical features of spinal and bulbar muscular atrophy. Brain 132(Pt 12):3242\u20133251. https:\/\/doi.org\/10.1093\/brain\/awp258","journal-title":"Brain"},{"issue":"1","key":"9052_CR6","doi-asserted-by":"publisher","first-page":"e84","DOI":"10.1212\/WNL.0000000000201342","volume":"100","author":"R Torii","year":"2023","unstructured":"Torii R, Hashizume A, Yamada S, Ito D, Kishimoto Y, Moriyoshi H, Inagaki T, Nakamura R, Nakamura T, Naoi T, Morita M, Katsuno M (2023) Clinical features of female carriers and prodromal male patients with spinal and bulbar muscular atrophy. Neurology 100(1):e84\u2013e93. https:\/\/doi.org\/10.1212\/WNL.0000000000201342","journal-title":"Neurology"},{"key":"9052_CR7","doi-asserted-by":"publisher","first-page":"873872","DOI":"10.1155\/2014\/873872","volume":"2014","author":"MO Harris-Love","year":"2014","unstructured":"Harris-Love MO, Fernandez-Rhodes L, Joe G, Shrader JA, Kokkinis A, La Pean Kirschner A, Auh S, Chen C, Li L, Levy E, Davenport TE, Di Prospero NA, Fischbeck KH (2014) Assessing function and endurance in adults with spinal and bulbar muscular atrophy: validity of the adult myopathy assessment tool. Rehabil Res Pract 2014:873872. https:\/\/doi.org\/10.1155\/2014\/873872","journal-title":"Rehabil Res Pract"},{"issue":"11","key":"9052_CR8","doi-asserted-by":"publisher","first-page":"1815","DOI":"10.1007\/s10072-016-2666-y","volume":"37","author":"G Querin","year":"2016","unstructured":"QuerinG, DaRe E, Martinelli I, Bello L, Bertolin C, Pareyson D, Mariotti C, Pegoraro E, Sorar\u00f9 G (2016) Validation of the Italian version of the SBMA Functional Rating Scale as outcome measure. Neurol Sci 37(11):1815\u20131821. https:\/\/doi.org\/10.1007\/s10072-016-2666-y","journal-title":"Neurol Sci"},{"issue":"2","key":"9052_CR9","doi-asserted-by":"publisher","first-page":"964","DOI":"10.1002\/mus.21077","volume":"38","author":"Y Takeuchi","year":"2008","unstructured":"Takeuchi Y, Katsuno M, Banno H, Suzuki K, Kawashima M, Atsuta N, Ito M, Watanabe H, Tanaka F, Sobue G (2008) Walking capacity evaluated by the 6-minute walk test in spinal and bulbar muscular atrophy. Muscle Nerve 38(2):964\u2013971. https:\/\/doi.org\/10.1002\/mus.21077","journal-title":"Muscle Nerve"},{"issue":"7","key":"9052_CR10","doi-asserted-by":"publisher","first-page":"554","DOI":"10.1016\/j.nmd.2015.03.008","volume":"25","author":"A Hashizume","year":"2015","unstructured":"Hashizume A, Katsuno M, Suzuki K, Banno H, Suga N, Mano T, Araki A, Hijikata Y, Grunseich C, Kokkinis A, Hirakawa A, Watanabe H, Yamamoto M, Fischbeck KH, Sobue G (2015) A functional scale for spinal and bulbar muscular atrophy: Cross-sectional and longitudinal study. Neuromuscul Disord 25(7):554\u2013562","journal-title":"Neuromuscul Disord"},{"issue":"Suppl 3","key":"9052_CR11","doi-asserted-by":"publisher","first-page":"III16","DOI":"10.1007\/BF03161085","volume":"246","author":"C Jenkinson","year":"1999","unstructured":"JenkinsonC, Fitzpatrick R, Brennan C, Bromberg M, Swash M (1999) Development and validation of a short measure of health status for individuals with amyotrophic lateral sclerosis\/motor neurone disease: the ALSAQ-40. J Neurol 246(Suppl 3):III16\u201321. https:\/\/doi.org\/10.1007\/BF03161085","journal-title":"J Neurol"},{"issue":"12","key":"9052_CR12","doi-asserted-by":"publisher","first-page":"e210088","DOI":"10.1212\/WNL.0000000000210088","volume":"103","author":"SB Huggett","year":"2024","unstructured":"Huggett SB, Tebbenkamp ATN, Rinaldi C, Jayaseelan D, Zampedri L, Blasi L, Fortuna A, Alqahtani A, Kokkinis A, Dahlqvist J, Fenu S, Cavalca E, Bertini A, Mariotti C, Grunseich C, Kawase T, Kishimoto Y, Yamada S, Katsuno M, Fratta P, Conte A, Sabatelli M, Soraru G, Vissing J, Kang M, Park JS, Pareyson D, Viglietta V (2024) Functional outcome measures to optimize drug development in spinal and bulbar muscular atrophy: results from a meta-analysis of the global SBMA dataset. Neurology 103(12):e210088. https:\/\/doi.org\/10.1212\/WNL.0000000000210088","journal-title":"Neurology"}],"container-title":["Neurological Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10072-026-09052-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10072-026-09052-x","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10072-026-09052-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,9]],"date-time":"2026-05-09T06:31:05Z","timestamp":1778308265000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10072-026-09052-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5]]},"references-count":12,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026,5]]}},"alternative-id":["9052"],"URL":"https:\/\/doi.org\/10.1007\/s10072-026-09052-x","relation":{},"ISSN":["1590-1874","1590-3478"],"issn-type":[{"value":"1590-1874","type":"print"},{"value":"1590-3478","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5]]},"assertion":[{"value":"26 February 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 April 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 May 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Informed consent was obtained from all subjects involved in the study","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"The authors report there are no competing interests to declare.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}],"article-number":"470"}}