{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T21:47:09Z","timestamp":1788385629790,"version":"build-2803163510"},"reference-count":32,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,2,20]],"date-time":"2025-02-20T00:00:00Z","timestamp":1740009600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100021856","name":"Ministero dell'Universit\u00e0 e della Ricerca","doi-asserted-by":"publisher","award":["PRIN 2017"],"award-info":[{"award-number":["PRIN 2017"]}],"id":[{"id":"10.13039\/501100021856","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100021856","name":"Ministero dell'Universit\u00e0 e della Ricerca","doi-asserted-by":"publisher","award":["2017SNW5MB"],"award-info":[{"award-number":["2017SNW5MB"]}],"id":[{"id":"10.13039\/501100021856","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010661","name":"Horizon 2020 Framework Programme","doi-asserted-by":"publisher","award":["101137074"],"award-info":[{"award-number":["101137074"]}],"id":[{"id":"10.13039\/100010661","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":[[2025,6]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>We aimed at evaluating the brain metabolic features of fused in sarcoma amyotrophic lateral sclerosis (<jats:italic>FUS<\/jats:italic>\u2010ALS) compared with sporadic ALS (sALS), using 2\u2010[fluorine\u201018] fluoro\u20102\u2010deoxy\u2010D\u2010glucose positron emission tomography (2\u2010[<jats:sup>18<\/jats:sup>F]FDG\u2010PET).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We employed the 2\u2010sample <jats:italic>t<\/jats:italic>\u2010test model of SPM12, implemented in MATLAB, to compare 12 <jats:italic>FUS<\/jats:italic>\u2010ALS cases with 40 healthy controls (HC) and 48 sALS, randomly collected from the series of patients who underwent brain 2\u2010[<jats:sup>18<\/jats:sup>F]FDG\u2010PET at the ALS Center of Turin (Italy) at diagnosis from 2009 to 2019. In the comparisons between cases and HC, we included age at PET and sex as covariates. Because <jats:italic>FUS<\/jats:italic>\u2010ALS usually shows early onset in spinal regions, in the comparison between <jats:italic>FUS<\/jats:italic>\u2010ALS and sALS, we included singularly the following covariates in a second step, to evaluate the determinants of eventual metabolic differences: age at PET, sex, and onset (spinal\/bulbar).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>sALS patients showed significant relative hypometabolism in bilateral fronto\u2010temporo\u2010occipital cortex and right insula as compared with <jats:italic>FUS<\/jats:italic>\u2010ALS. After adjusting for age, the relative hypometabolism remained in the bilateral precentral gyrus and in the right middle and inferior temporal gyrus. As compared with HC, <jats:italic>FUS<\/jats:italic> patients displayed a significant relative hypermetabolism in the pontobulbar region and right cerebellar tonsil, dentate nucleus, and uvula, while sALS showed relative hypometabolism in bilateral frontal and occipital cortices and in left temporal and parietal regions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Patients with <jats:italic>FUS<\/jats:italic>\u2010ALS show relative preservation of motor cortex metabolism compared with those with sALS, possibly reflecting the prevalence of lower motor neuron impairment in their phenotype. Prospective studies are necessary to investigate the possible role of 2\u2010[<jats:sup>18<\/jats:sup>F]FDG\u2010PET as a biomarker to track disease spreading in clinical trials. ANN NEUROL 2025;97:1134\u20131143<\/jats:p><\/jats:sec>","DOI":"10.1002\/ana.27201","type":"journal-article","created":{"date-parts":[[2025,2,20]],"date-time":"2025-02-20T11:19:27Z","timestamp":1740050367000},"page":"1134-1143","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Brain Metabolic Features of <i>FUS<\/i>\u2010<scp>ALS<\/scp>: A 2\u2010[<scp><sup>18<\/sup>F<\/scp>]<scp>FDG<\/scp>\u2010<scp>PET<\/scp> Study"],"prefix":"10.1002","volume":"97","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5876-4079","authenticated-orcid":false,"given":"Antonio","family":"Canosa","sequence":"first","affiliation":[{"name":"ALS Center, \u2018Rita Levi Montalcini\u2019 Department of Neuroscience University of Turin  Turin Italy"},{"name":"Azienda Ospedaliero\u2010Universitaria Citt\u00e0 della Salute e della Scienza di Torino, Neurology Unit 1U  Turin Italy"},{"name":"Institute of Cognitive Sciences and Technologies, CNR  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