{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T17:19:52Z","timestamp":1788283192994,"version":"build-2803163510"},"reference-count":17,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2026,2,22]],"date-time":"2026-02-22T00:00:00Z","timestamp":1771718400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,2,22]],"date-time":"2026-02-22T00:00:00Z","timestamp":1771718400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["U01NS101944"],"award-info":[{"award-number":["U01NS101944"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Targeted therapies for facioscapulohumeral muscular dystrophy (FSHD) are progressing through clinical trials. Electrical impedance myography (EIM) provides a noninvasive biomarker of muscle composition that may be valuable especially in early phase trials. This study evaluated EIM data from a multicenter FSHD cohort over 24\u2009months.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Adult patients with FSHD at 8 sites underwent EIM in 6 muscles bilaterally (deltoid, biceps, triceps, vastus lateralis, tibialis anterior, and medial gastrocnemius). EIM outcomes phase and reactance (50 and 100\u2009kHz [kilohertz] frequencies) and 50 of 211\u2009kHz phase ratio were evaluated for reliability, correlations with clinical measures, and sensitivity to change.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      One hundred fifty\u2010seven patients (53% male patients) were included. Test\u2013retest reliability was excellent for all EIM outcomes (intraclass correlation coefficient [ICC]\u2009\u22650.94). Phase outcomes strongly correlated with the FSHD\u2010composite outcome measure (FSHD\u2010COM;\n                      <jats:italic>r<\/jats:italic>\n                      \u2009\u2264\u2009\u22120.69) and Motor Function Measure Domain 1 (MFM1;\n                      <jats:italic>r<\/jats:italic>\n                      \u2009\u2265\u20090.75); reactance outcomes exhibited moderate correlations with the FSHD\u2010COM (\n                      <jats:italic>r<\/jats:italic>\n                      \u2009\u2265\u2009\u20130.41) and MFM1 (\n                      <jats:italic>r<\/jats:italic>\n                      \u2009\u2264\u20090.44). Mean declines in phase and phase ratio were apparent at 12\u2009months (eg, \u20130.25, 95% confidence interval [CI]\u2009=\u2009\u20130.45 to \u20130.05 at 50\u2009kHz), and further progressed through 24\u2009months (\u20130.66, 95% CI\u2009=\u2009\u20130.92 to \u20130.40] at 50\u2009kHz and \u20130.65 [95% CI\u2009=\u2009\u20130.87 to \u20130.44] at 100\u2009kHz; both\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.0001). Reactance changes were smaller and not significant: \u20130.21 (95% CI\u2009=\u2009\u20130.44 to 0.02) at 50\u2009kHz and \u20130.13 (95% CI\u2009=\u2009\u20130.35 to 0.10) at 100\u2009kHz.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>EIM phase outcomes are reliable, valid, and sensitive to change over 12 to 24\u2009months, supporting their potential utility as biomarkers in FSHD clinical trials. ANN NEUROL 2026;99:1545\u20131554<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ana.78182","type":"journal-article","created":{"date-parts":[[2026,2,23]],"date-time":"2026-02-23T04:05:20Z","timestamp":1771819520000},"page":"1545-1554","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Electrical Impedance Myography Detects Disease Progression over 12 to 24\u2009Months in Facioscapulohumeral Muscular Dystrophy"],"prefix":"10.1002","volume":"99","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8487-9478","authenticated-orcid":false,"given":"Karlien","family":"Mul","sequence":"first","affiliation":[{"name":"Department of Neurology Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael P.","family":"McDermott","sequence":"additional","affiliation":[{"name":"Department of Biostatistics and Computational Biology University of Rochester Medical Center  Rochester NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell J.","family":"Butterfield","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Utah  Salt Lake City UT"},{"name":"Department of Pediatrics University of Utah  Salt Lake City UT"},{"name":"Department of Physical Therapy and Athletic Training University of Utah  Salt Lake City UT"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bakri","family":"Elsheikh","sequence":"additional","affiliation":[{"name":"Department of Neurology The Ohio State University  Columbus OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kate","family":"Eichinger","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Rochester Medical Center  Rochester NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas E.","family":"Johnson","sequence":"additional","affiliation":[{"name":"Center for Inherited Muscle Research, Department of Neurology Virginia Commonwealth University  Richmond VA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2558-7798","authenticated-orcid":false,"given":"Doris G.","family":"Leung","sequence":"additional","affiliation":[{"name":"Center for Genetic Muscle Disorders, Department of Neurology at the Johns Hopkins University School of Medicine Kennedy Krieger Institute  Baltimore MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha","family":"LoRusso","sequence":"additional","affiliation":[{"name":"Department of Neurology The Ohio State University  Columbus OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leann","family":"Lewis","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Rochester Medical Center  Rochester NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William B.","family":"Martens","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Rochester Medical Center  Rochester NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Perry B.","family":"Shieh","sequence":"additional","affiliation":[{"name":"Department of Neurology at the David Geffen School of Medicine University of California Los Angeles  Los Angeles CA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5079-1416","authenticated-orcid":false,"given":"Leo H.","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Washington  Seattle WA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Walker","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Kansas Medical Center  Kansas City KS"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1394-1494","authenticated-orcid":false,"given":"Rabi","family":"Tawil","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Rochester Medical Center  Rochester NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey M.","family":"Statland","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Kansas Medical Center  Kansas City KS"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"ReSolve Investigators of the 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