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Neuroimaging demonstrated right anterior temporal atrophy before cognitive symptoms, and prosopagnosia represented the earliest manifestation of rtvFTD. Molecular analysis revealed reduced levels of correctly spliced\n                    <jats:italic>TBK1<\/jats:italic>\n                    transcripts, consistent with haploinsufficiency. Given the shared involvement of TDP\u201043 pathology in both PLS and rtvFTD, this case indicates\n                    <jats:italic>TBK1<\/jats:italic>\n                    dysfunction as a fundamental genetic factor underlying the coexistence of these phenotypes, underscoring the clinical value of early neuroimaging and genetic evaluation.\n                  <\/jats:p>","DOI":"10.1002\/acn3.70329","type":"journal-article","created":{"date-parts":[[2026,2,6]],"date-time":"2026-02-06T04:20:15Z","timestamp":1770351615000},"page":"1073-1077","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["<scp>\n                      <i>TBK<\/i>\n                    <\/scp>\n                    <i>1<\/i>\n                    \u2010Associated Primary Lateral Sclerosis Followed by Right Temporal Variant Frontotemporal Dementia"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7559-8284","authenticated-orcid":false,"given":"Tomoyasu","family":"Matsubara","sequence":"first","affiliation":[{"name":"Department of Neurology Tokushima University Hospital  Tokushima Japan"},{"name":"Department of Clinical Neuroscience and Therapeutics, Graduate School of Biomedical and Health Sciences Hiroshima University  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naoki","family":"Kihara","sequence":"additional","affiliation":[{"name":"Department of Neurology Tokushima University Hospital  Tokushima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7587-5168","authenticated-orcid":false,"given":"Satoko","family":"Miyatake","sequence":"additional","affiliation":[{"name":"Department of Clinical Genetics Yokohama City University Hospital  Yokohama Japan"},{"name":"Department of Human Genetics Yokohama 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of Clinical and Translational 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