{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T21:11:19Z","timestamp":1762981879001,"version":"3.41.2"},"reference-count":56,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,7,10]],"date-time":"2024-07-10T00:00:00Z","timestamp":1720569600000},"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":["P30\u2010AG062677","U54\u2010NS100693","P01\u2010AG03949","R01\u2010AG062348","1U19AG063911","RF1\u2010NS123052","R01\u2010NS121125","FAIN: U19AG063911"],"award-info":[{"award-number":["P30\u2010AG062677","U54\u2010NS100693","P01\u2010AG03949","R01\u2010AG062348","1U19AG063911","RF1\u2010NS123052","R01\u2010NS121125","FAIN: U19AG063911"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Brain Pathology"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by upper and lower motor neuron signs. There are, however, cases where upper motor neurons (UMNs) are predominantly affected, leading to clinical presentations of UMN\u2010dominant ALS or primary lateral sclerosis. Furthermore, cases exhibiting an UMN\u2010predominant pattern of motor neuron disease (MND) presenting with corticobasal syndrome (CBS) have been sparsely reported. This study aims to clarify the clinicopathological features of patients with UMN\u2010predominant MND. We reviewed 24 patients with UMN\u2010predominant MND with TDP\u201043 pathology in the presence or absence of frontotemporal lobar degeneration. Additionally, we reviewed the medical records of patients with pathologically\u2010confirmed corticobasal degeneration (CBD) who received a final clinical diagnosis of CBS (<jats:italic>n<\/jats:italic>\u2009=\u200910) and patients with pathologically\u2010confirmed progressive supranuclear palsy (PSP) who received a final clinical diagnosis of PSP syndrome (<jats:italic>n<\/jats:italic>\u2009=\u200910). Of 24 UMN\u2010predominant MND patients, 20 had a clinical diagnosis of an atypical parkinsonian disorder, including CBS (<jats:italic>n\u2009=<\/jats:italic>\u200911) and PSP syndrome (<jats:italic>n\u2009=<\/jats:italic>\u20098). Only two patients had antemortem diagnoses of motor neuron disease. UMN\u2010predominant MND patients with CBS less frequently exhibited apraxia than those with CBD, and they were less likely to meet clinical criteria for possible or probable CBS. Similarly, UMN\u2010predominant MND patients with PSP syndrome less often met clinical criteria for probable PSP than PSP patients with PSP syndrome. Our findings suggest that UMN\u2010predominant MND can mimic atypical parkinsonism, and should be considered in the differential diagnosis of CBS and PSP syndrome, in particular when criteria are not met.<\/jats:p>","DOI":"10.1111\/bpa.13286","type":"journal-article","created":{"date-parts":[[2024,7,11]],"date-time":"2024-07-11T05:23:36Z","timestamp":1720675416000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Upper motor neuron\u2010predominant motor neuron disease presenting as atypical parkinsonism: A clinicopathological study"],"prefix":"10.1111","volume":"35","author":[{"given":"Aya","family":"Murakami","sequence":"first","affiliation":[{"name":"Department of Neuroscience Mayo Clinic  Jacksonville Florida USA"},{"name":"Department of Neurology Kansai Medical University  Osaka 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USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin F.","family":"Bieniek","sequence":"additional","affiliation":[{"name":"Department of Pathology &amp; Laboratory Medicine University of Texas Health Science Center San Antonio  Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroaki","family":"Sekiya","sequence":"additional","affiliation":[{"name":"Department of Neuroscience Mayo Clinic  Jacksonville Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mariely","family":"DeJesus\u2010Hernandez","sequence":"additional","affiliation":[{"name":"Department of Neuroscience Mayo Clinic  Jacksonville Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"NiCole A.","family":"Finch","sequence":"additional","affiliation":[{"name":"Department of Neuroscience Mayo Clinic  Jacksonville Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marka","family":"van 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