{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T15:42:46Z","timestamp":1787499766438,"version":"build-2736575974"},"reference-count":21,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2024,10,5]],"date-time":"2024-10-05T00:00:00Z","timestamp":1728086400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","award":["RF\u20102016\u201002364123"],"award-info":[{"award-number":["RF\u20102016\u201002364123"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disord Clin Pract"],"published-print":{"date-parts":[[2024,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Pediatric\u2010onset Huntington's disease (POHD) exhibits a phenotype different from adult\u2010onset HD (AOHD), with hypokinetic movement disorders (eg, rigidity, bradykinesia, and dystonia) rather than chorea typical of AOHD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>The aim was to identify pathophysiology\u2010based biomarkers specific to POHD (\u226560 CAG repeats).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Simultaneous hybrid imaging using [\n                      <jats:sup>18<\/jats:sup>\n                      F]fluoro\u20102\u2010deoxy\u2010\n                      <jats:sc>d<\/jats:sc>\n                      \u2010glucose (FDG) positron emission tomography plus magnetic resonance imaging (FDG\u2010PET\/MRI) and clinical assessment using standardized Huntington's disease (HD) scales were employed. Exploratory longitudinal analyses were also performed.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Striatal volume loss was remarkable and more severe in POHD (n\u2009=\u20095) than in AOHD (n\u2009=\u200914). Widespread, significantly altered glucose metabolism occurred in several different POHD cortical areas and thalamus, but not AOHD cortex, consistent with differences in clinical progression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>POHD patients' brains exhibited distinct morphologic and metabolic traits compared to AOHD patients\u2019 brains, with longitudinal changes mirroring clinical progression. Hybrid FDG\u2010PET\/MRI highlighted a variable regional brain dysfunction in vivo, as a biological consequence of highly expanded CAG repeats. Findings provide further evidence that POHD is a distinct disease from AOHD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mdc3.14223","type":"journal-article","created":{"date-parts":[[2024,10,5]],"date-time":"2024-10-05T03:45:28Z","timestamp":1728099928000},"page":"1592-1597","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Pediatric Huntington Disease Brains Have Distinct Morphologic and Metabolic Traits: the\n                    <scp>RAREST<\/scp>\n                    \u2010\n                    <scp>JHD<\/scp>\n                    Study"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2030-5552","authenticated-orcid":false,"given":"Maria Eugenia","family":"Caligiuri","sequence":"first","affiliation":[{"name":"Department of Medical and Surgical Sciences, Neuroscience Research Centre University Magna Graecia  Catanzaro 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Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5321-8626","authenticated-orcid":false,"given":"Umberto","family":"Sabatini","sequence":"additional","affiliation":[{"name":"Department of Medical and Surgical Sciences, Neuroscience Research Centre University Magna Graecia  Catanzaro Italy"},{"name":"Department of Medical and Surgical Sciences University Magna Graecia  Catanzaro Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7397-1727","authenticated-orcid":false,"given":"Ferdinando","family":"Squitieri","sequence":"additional","affiliation":[{"name":"Centre for Neurological Rare Diseases (CMNR) Fondazione Lega Italiana Ricerca Huntington (LIRH)  Rome Italy"},{"name":"Huntington and Rare Diseases Unit Fondazione IRCCS Casa Sollievo della Sofferenza (CSS) Hospital  San Giovanni Rotondo 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