{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T16:02:47Z","timestamp":1780588967342,"version":"3.54.1"},"reference-count":15,"publisher":"BMJ","issue":"2","funder":[{"DOI":"10.13039\/501100002426","name":"Fondazione Telethon","doi-asserted-by":"publisher","award":["GUP15009"],"award-info":[{"award-number":["GUP15009"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]},{"name":"This work was partially supported by the Italian Ministry of Health (RRC)."}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"accepted":{"date-parts":[[2022,8,31]]},"published-print":{"date-parts":[[2023,2]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Spino-bulbar muscular atrophy (SBMA), caused by a CAG repeat expansion in the androgen receptor gene, affects adult men and results in muscle atrophy and weakness in the bulbar and limb muscles and signs of partial androgen insensitivity. During the COVID-19 pandemic, outpatients\u2019 visits have been reduced to preserve safety of frail patients, and telehealth was largely employed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>From April to November 2020, we monitored 12 patients with SBMA with telehealth and administered remotely two clinical scales currently used for SBMA: Adult Myopathy Assessment Tool (AMAT) and SBMA-Functional Rating Scale (SBMA-FRS). We compared results with previous and subsequent in-person visits\u2019 scores, and assessed the longitudinal changes in AMAT and SBMA-FRS scores during 7 years through the repeated measures analysis of variance (ANOVA).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Repeated measures ANOVA of AMAT scores collected during 7\u2009years and including tele-AMAT evaluation showed a steady mean decline of 1\u20132 points per year. A similar trend of SBMA-FRS scores, with a mean decline per year of about 1 point, was observed. There was no relevant deviation from the model prediction.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Our data show that telehealth is a valid tool to monitor patients with SBMA: AMAT and SBMA-FRS scales can be effectively, reliably and easily administered remotely.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/jnnp-2022-329616","type":"journal-article","created":{"date-parts":[[2022,9,22]],"date-time":"2022-09-22T13:21:16Z","timestamp":1663852876000},"page":"161-161","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["Reliable virtual clinical assessment in spino-bulbar muscular atrophy (SBMA)"],"prefix":"10.1136","volume":"94","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5233-6580","authenticated-orcid":false,"given":"Silvia","family":"Fenu","sequence":"first","affiliation":[{"name":"Department of Clinical Neurosciences, Unit of Rare Neurodegenerative and Neurometabolic Diseases, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5550-3412","authenticated-orcid":false,"given":"Irene","family":"Tramacere","sequence":"additional","affiliation":[{"name":"Department of Research and Clinical Development, Scientific Directorate, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"De Giorgi","sequence":"additional","affiliation":[{"name":"Unit of Informative Services, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6854-765X","authenticated-orcid":false,"given":"Davide","family":"Pareyson","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurosciences, Unit of Rare Neurodegenerative and Neurometabolic Diseases, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2022,9,22]]},"reference":[{"key":"2026060408300921000_94.2.161.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurol.2017.03.019"},{"key":"2026060408300921000_94.2.161.2","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-018-8968-7"},{"key":"2026060408300921000_94.2.161.3","first-page":"1","article-title":"Assessing function and endurance in adults with spinal and bulbar muscular atrophy: validity of the adult myopathy assessment tool","volume":"2014","author":"Harris-Love","year":"2014","journal-title":"Rehabil Res Pract"},{"key":"2026060408300921000_94.2.161.4","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2015.03.008"},{"key":"2026060408300921000_94.2.161.5","doi-asserted-by":"publisher","DOI":"10.1007\/s12031-015-0704-5"},{"key":"2026060408300921000_94.2.161.6","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-018-0918-z"},{"key":"2026060408300921000_94.2.161.7","doi-asserted-by":"publisher","DOI":"10.1016\/S0090-4295(99)80447-6"},{"key":"2026060408300921000_94.2.161.8","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ijir.3900472"},{"key":"2026060408300921000_94.2.161.9","doi-asserted-by":"publisher","DOI":"10.1007\/s10072-021-05252-9"},{"key":"2026060408300921000_94.2.161.10","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000009566"},{"key":"2026060408300921000_94.2.161.11","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26881"},{"key":"2026060408300921000_94.2.161.12","doi-asserted-by":"publisher","DOI":"10.3389\/fgene.2021.735538"},{"key":"2026060408300921000_94.2.161.13","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-021-02165-w"},{"key":"2026060408300921000_94.2.161.14","doi-asserted-by":"publisher","DOI":"10.1111\/j.1529-8027.2011.00350.x"},{"key":"2026060408300921000_94.2.161.15","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000006323"}],"container-title":["Journal of Neurology, Neurosurgery &amp; Psychiatry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jnnp-2022-329616","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T15:30:17Z","timestamp":1780587017000},"score":1,"resource":{"primary":{"URL":"https:\/\/jnnp.bmj.com\/lookup\/doi\/10.1136\/jnnp-2022-329616"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,9,22]]},"references-count":15,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2023,1,11]]},"published-print":{"date-parts":[[2023,2]]}},"alternative-id":["10.1136\/jnnp-2022-329616"],"URL":"https:\/\/doi.org\/10.1136\/jnnp-2022-329616","relation":{},"ISSN":["0022-3050","1468-330X"],"issn-type":[{"value":"0022-3050","type":"print"},{"value":"1468-330X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,9,22]]}}}