{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T16:07:31Z","timestamp":1790179651116,"version":"4.1.0"},"reference-count":36,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2024,4,30]],"date-time":"2024-04-30T00:00:00Z","timestamp":1714435200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01\u2010NS89757"],"award-info":[{"award-number":["R01\u2010NS89757"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Primary lateral sclerosis (PLS) is a neurodegenerative disorder that primarily affects the central motor system. In rare cases, clinical features of PLS may overlap with those of progressive supranuclear palsy (PSP). We investigate neuroimaging features that can help distinguish PLS with overlapping features of PSP (PLS\u2010PSP) from PSP.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Six patients with PLS\u2010PSP were enrolled between 2019 and 2023. We compared their clinical and neuroimaging characteristics with 18 PSP\u2013Richardson syndrome (PSP\u2010RS) patients and 20 healthy controls. Magnetic resonance imaging, <jats:sup>18<\/jats:sup>F\u2010flortaucipir positron emission tomography (PET), quantitative susceptibility mapping, and diffusion tensor imaging tractography (DTI) were performed to evaluate eight brain regions of interest. Area under the receiver operating characteristic curve (AUROC) was calculated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Five of the six PLS\u2010PSP patients (83.3%) were male. Median age at symptom onset was 61.5 (52.5\u201363) years, and all had mixed features of PLS and PSP. Volumes of the pallidum, caudate, midbrain, and cerebellar dentate were smaller in PSP\u2010RS than PLS\u2010PSP, providing good discrimination (AUROC\u2009=\u20090.75 for all). The susceptibilities in pallidum, midbrain, and cerebellar dentate were greater in PSP\u2010RS compared to PLS\u2010PSP, providing excellent discrimination (AUROC \u2265\u20090.90 for all). On DTI, fractional anisotropy (FA) in the posterior limb of the internal capsule from the corticospinal tract was lower in PLS\u2010PSP compared to PSP\u2010RS (AUROC\u2009=\u20090.86), but FA in the superior cerebellar peduncle was lower in PSP\u2010RS (AUROC\u2009=\u20090.95). Pallidum flortaucipir PET uptake was greater in PSP\u2010RS compared to PLS\u2010PSP (AUROC\u2009=\u20090.74).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Regional brain volume, tractography, and magnetic susceptibility, but not tau\u2010PET, are useful in distinguishing PLS\u2010PSP from PSP.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.16320","type":"journal-article","created":{"date-parts":[[2024,4,30]],"date-time":"2024-04-30T12:39:21Z","timestamp":1714480761000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Clinical and neuroimaging characteristics of primary lateral sclerosis with overlapping features of progressive supranuclear palsy"],"prefix":"10.1111","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3520-5212","authenticated-orcid":false,"given":"Negin","family":"Badihian","sequence":"first","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2170-6662","authenticated-orcid":false,"given":"Rodolfo G.","family":"Gatto","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryota","family":"Satoh","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Farwa","family":"Ali","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9697-0084","authenticated-orcid":false,"given":"Heather M.","family":"Clark","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nha Trang Thu","family":"Pham","sequence":"additional","affiliation":[{"name":"Department of Radiology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer L.","family":"Whitwell","sequence":"additional","affiliation":[{"name":"Department of Radiology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2930-8634","authenticated-orcid":false,"given":"Keith A.","family":"Josephs","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,4,30]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2019-322,541"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.24990"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm13020578"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.26987"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awr178"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2011.107"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.parkreldis.2011.05.013"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nicl.2022.103030"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.2006.00767.x"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.29613"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nicl.2019.102152"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-007-0234-5"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e32833be924"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.15603"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.17879\/freeneuropathology-2020-2639"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/115.2.495"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1532-5415.2005.53221.x"},{"key":"e_1_2_10_19_1","volume-title":"Neuropsychological assessment","author":"Lezak MD","year":"2004"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/mdc3.13010"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.21198"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nicl.2022.103161"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.3390\/cells12242776"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ncl.2015.07.007"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2024.120509"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2014.12.043"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2017.12.040"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mri.2019.01.018"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13311-018-0663-y"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0080713"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2018.05.027"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2018-318,788"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.jnen.0000218446.38158.61"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.18383\/j.tom.2015.00136"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.14.13459"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.26844"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.25060"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.16320","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,6]],"date-time":"2024-07-06T06:56:58Z","timestamp":1720249018000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.16320"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,30]]},"references-count":36,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2024,8]]}},"alternative-id":["10.1111\/ene.16320"],"URL":"https:\/\/doi.org\/10.1111\/ene.16320","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,30]]},"assertion":[{"value":"2024-02-14","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-04-11","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-04-30","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e16320"}}