{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T21:04:23Z","timestamp":1773954263246,"version":"3.50.1"},"reference-count":33,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,5,25]],"date-time":"2024-05-25T00:00:00Z","timestamp":1716595200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["U54NS115052"],"award-info":[{"award-number":["U54NS115052"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009633","name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development","doi-asserted-by":"publisher","award":["P50HD103538"],"award-info":[{"award-number":["P50HD103538"]}],"id":[{"id":"10.13039\/100009633","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The most common manifestation of X\u2010linked adrenoleukodystrophy (ALD) is a slowly progressive myeloneuropathy, which leads to imbalance and gait disturbances. The variable progression of the disease complicates evaluation of its progression rate. Wearable sensors allow for easy and frequent balance and gait collection. This study reports baseline data from a longitudinal study on the quantitative assessment of balance and gait with wearable sensors and their clinical relevance.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data were collected from adult patients in two institutions. Postural body sway and gait parameters were measured using accelerometers. Disease severity was measured by the Expanded Disability Severity Scale (EDSS). Falling frequency and quality of life (QOL) were collected in men. The relationship between sway and gait variables and EDSS score, participants' use of a walking aid, and falling frequency was evaluated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>One hundred twenty individuals with ALD were included. Sway variables significantly differentiate participants' assistive device use. Sway and gait variables were correlated to the EDSS in both sexes. Both gait speed and sway were correlated with falling frequency in men from one institution. Select QOL subscores were correlated with the EDSS in males from one institution. Accelerometry generated comparable results across sites.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>This study confirms the clinical correlation between spinal cord disease and imbalance and gait in ALD. For the first time, this study shows clinically meaningful relationships for sway and gait with use of an assistive device, falling frequency and QOL. Wearable accelerometers are a valid means to measure sway and gait in ALD. These measures are promising outcomes for clinical trial designs to assess myeloneuropathy in ALD and to monitor disease progression in individuals.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jimd.12753","type":"journal-article","created":{"date-parts":[[2024,5,25]],"date-time":"2024-05-25T12:28:59Z","timestamp":1716640139000},"page":"1336-1347","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["International validation of meaningfulness of postural sway and gait to assess myeloneuropathy in adults with adrenoleukodystrophy"],"prefix":"10.1002","volume":"47","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-7973-2724","authenticated-orcid":false,"given":"Hemmo A. F.","family":"Yska","sequence":"first","affiliation":[{"name":"Emma Children's Hospital, Department of Neurology and Pediatric Neurology, Amsterdam Leukodystrophy Center, Amsterdam University Medical Centers University of Amsterdam  Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bela R.","family":"Turk","sequence":"additional","affiliation":[{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"},{"name":"Department of Neurology Johns Hopkins School of Medicine  Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ali","family":"Fatemi","sequence":"additional","affiliation":[{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"},{"name":"Department of Neurology Johns Hopkins School of Medicine  Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jordan","family":"Goodman","sequence":"additional","affiliation":[{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marije","family":"Voermans","sequence":"additional","affiliation":[{"name":"Emma Children's Hospital, Department of Neurology and Pediatric Neurology, Amsterdam Leukodystrophy Center, Amsterdam University Medical Centers University of Amsterdam  Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dan","family":"Amos","sequence":"additional","affiliation":[{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Man","family":"Amanat","sequence":"additional","affiliation":[{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephanie","family":"van de Stadt","sequence":"additional","affiliation":[{"name":"Emma Children's Hospital, Department of Neurology and Pediatric Neurology, Amsterdam Leukodystrophy Center, Amsterdam University Medical Centers University of Amsterdam  Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5346-8497","authenticated-orcid":false,"given":"Marc","family":"Engelen","sequence":"additional","affiliation":[{"name":"Emma Children's Hospital, Department of Neurology and Pediatric Neurology, Amsterdam Leukodystrophy Center, Amsterdam University Medical Centers University of Amsterdam  Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amena","family":"Smith\u2010Fine","sequence":"additional","affiliation":[{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"},{"name":"Department of Neurology Johns Hopkins School of Medicine  Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer","family":"Keller","sequence":"additional","affiliation":[{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"},{"name":"Department of Physical Medicine &amp; Rehabilitation Johns Hopkins School of Medicine  Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,5,25]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750\u20101172\u20107\u201051"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010019\u20101008\u20106"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/59.2.89"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12457"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/brb3.2878"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11910\u2010014\u20100486\u20100"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awy299"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.51509"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.4172\/2155\u20109538.S1\u2010007"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1186\/1743\u20100003\u20109\u201059"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1177\/1545968312441682"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.63.1.74"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2020.00786"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/milmed\/usx162"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humov.2021.102881"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.4085\/1062\u20106050\u2010518\u201017"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gaitpost.2012.04.010"},{"key":"e_1_2_10_19_1","volume-title":"Mobility Lab Whitepaper","author":"APDM","year":"2019"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.26849"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25120"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.apmr.2017.04.004"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1097\/mrr.0000000000000205"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010018\u20100848\u20109"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.13\u20100287"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/00008483\u2010200603000\u201000011"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2018.02.073"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2021.684102"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.5535\/arm.2012.36.1.112"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0749-0690(18)30194-0"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gaitpost.2006.07.005"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12901\u2010017\u20100034\u2010z"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1586\/17434440.2016.1153421"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gaitpost.2023.09.012"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jimd.12753","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,25]],"date-time":"2024-11-25T06:43:16Z","timestamp":1732516996000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jimd.12753"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,25]]},"references-count":33,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2024,11]]}},"alternative-id":["10.1002\/jimd.12753"],"URL":"https:\/\/doi.org\/10.1002\/jimd.12753","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5,25]]},"assertion":[{"value":"2024-01-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-06","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-25","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}