{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,30]],"date-time":"2025-12-30T23:37:53Z","timestamp":1767137873008,"version":"build-2238731810"},"reference-count":22,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,7,28]],"date-time":"2023-07-28T00:00:00Z","timestamp":1690502400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2023,10]]},"abstract":"Abstract<\/jats:title>\n \n Introduction\/Aims<\/jats:title>\n \n The periodic paralyses are muscle channelopathies: hypokalemic periodic paralysis (\n CACNA1S<\/jats:italic>\n and\n SCN4A<\/jats:italic>\n variants), hyperkalemic periodic paralysis (\n SCN4A<\/jats:italic>\n variants), and Andersen\u2010Tawil syndrome (\n KCNJ2<\/jats:italic>\n ). Both episodic weakness and disabling fixed weakness can occur. Little literature exists on magnetic resonance imaging (MRI) in muscle channelopathies. We undertake muscle MRI across all subsets of periodic paralysis and correlate with clinical features.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n A total of 45 participants and eight healthy controls were enrolled and underwent T1\u2010weighted and short\u2010tau\u2010inversion\u2010recovery (STIR) MRI imaging of leg muscles. Muscles were scored using the modified Mercuri Scale.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n A total of 17 patients had\n CACNA1S<\/jats:italic>\n variants, 16\n SCN4A,<\/jats:italic>\n and 12\n KCNJ2<\/jats:italic>\n . Thirty\u2010one (69%) had weakness, and 9 (20%) required a gait\u2010aid\/wheelchair. A total of 78% of patients had intramuscular fat accumulation on MRI. Patients with\n SCN4A<\/jats:italic>\n variants were most severely affected. In\n SCN4A,<\/jats:italic>\n the anterior thigh and posterior calf were more affected, in contrast to the posterior thigh and posterior calf in\n KCNJ2<\/jats:italic>\n . We identified a pattern of peri\u2010tendinous STIR hyperintensity in nine patients. There were moderate correlations between Mercuri, STIR scores, and age. Intramuscular fat accumulation was seen in seven patients with no fixed weakness.\n <\/jats:p>\n <\/jats:sec>\n \n Discussion<\/jats:title>\n We demonstrate a significant burden of disease in patients with periodic paralyses. MRI intramuscular fat accumulation may be helpful in detecting early muscle involvement, particularly in those without fixed weakness. Longitudinal studies are needed to assess the role of muscle MRI in quantifying disease progression over time and as a potential biomarker in clinical trials.<\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/mus.27947","type":"journal-article","created":{"date-parts":[[2023,7,29]],"date-time":"2023-07-29T02:11:40Z","timestamp":1690596700000},"page":"439-450","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Muscle\n MRI<\/scp>\n in periodic paralysis shows myopathy is common and correlates with intramuscular fat accumulation"],"prefix":"10.1002","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4944-0061","authenticated-orcid":false,"given":"Vinojini","family":"Vivekanandam","sequence":"first","affiliation":[{"name":"Queen Square Centre for Neuromuscular Diseases UCL Queen Square Institute of Neurology London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karen","family":"Suetterlin","sequence":"additional","affiliation":[{"name":"Queen Square Centre for Neuromuscular Diseases UCL Queen Square Institute of Neurology London UK"},{"name":"AGE Research Group, NIHR Newcastle Biomedical Research Centre Newcastle upon Tyne Hospitals NHS Foundation Trust and Newcastle University Newcastle upon Tyne UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emma","family":"Matthews","sequence":"additional","affiliation":[{"name":"Atkinson\u2010Morley Neuromuscular Centre, Department of Neurology St George's University Hospitals NHS Foundation Trust, and Molecular and Clinical Sciences Research Institute, St George's University of London London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Thornton","sequence":"additional","affiliation":[{"name":"Neuroradiological Academic Unit UCL Queen Square Institute of Neurology, UCL London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dipa","family":"Jayaseelan","sequence":"additional","affiliation":[{"name":"Queen Square Centre for Neuromuscular Diseases UCL Queen Square Institute of Neurology London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sachit","family":"Shah","sequence":"additional","affiliation":[{"name":"Neuroradiological Academic Unit UCL Queen Square Institute of Neurology, UCL London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jasper M.","family":"Morrow","sequence":"additional","affiliation":[{"name":"Queen Square Centre for Neuromuscular Diseases UCL Queen Square Institute of Neurology London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tarek","family":"Yousry","sequence":"additional","affiliation":[{"name":"Neuroradiological Academic Unit UCL Queen Square Institute of Neurology, UCL London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael G.","family":"Hanna","sequence":"additional","affiliation":[{"name":"Queen Square Centre for Neuromuscular Diseases UCL Queen Square Institute of Neurology London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,7,28]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2023.01.007"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474\u20104422(15)00242\u20102"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2018.00456"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp\u20102018\u2010320198"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2018.06.008"},{"key":"e_1_2_11_7_1","first-page":"109","article-title":"Imaging alterations in skeletal muscle channelopathies: a study in 15 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