{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T20:51:30Z","timestamp":1774471890894,"version":"3.50.1"},"reference-count":15,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,7,28]],"date-time":"2021-07-28T00:00:00Z","timestamp":1627430400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP20ek0109391"],"award-info":[{"award-number":["JP20ek0109391"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP20ek0109392"],"award-info":[{"award-number":["JP20ek0109392"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2021,10]]},"abstract":"<jats:p>The hot cross bun (HCB) sign encompasses a cross\u2010shaped hyperintensity area in the pons on axial T2\u2010weighted magnetic resonance imaging (MRI). The HCB sign is characteristic of multiple system atrophy (MSA) and has occasionally been observed in other neurological disorders. Here, we report an autopsied case of corticobasal degeneration (CBD) that showed the HCB sign. A female patient presented with progressive gait disturbance and cognitive impairment at the age of 60\u2009years. A neurological examination revealed dysarthria, muscle rigidity of the limbs, akinesia, truncal ataxia, urinary incontinence, and dementia. The HCB sign was observed on a brain MRI at the age of 65\u2009years, and a clinical diagnosis of possible MSA was made. She died of pneumonia at the age of 67\u2009years. A postmortem observation, provided neuropathological findings characteristic of CBD, including the presence of astrocytic plaques, pretangles, neuropil threads, and ballooned neurons in association with four\u2010repeat\u2010tau aggregation. Interestingly, the pons displayed severe neuronal loss and astrogliosis that were prominent in the pontine and raphe nuclei. Myelin sheath depletion was prominent in the transverse fibers of the pontine base and the myelinated fibers of the pontine tegmentum in contrast to relative sparing of the pontine corticospinal tract and medial lemniscus. The cerebellar dentate nucleus exhibited neuronal loss and grumose degeneration. Western blot analysis of sarkosyl\u2010insoluble fractions from brain tissue lysates using an anti\u2010phosphorylated tau antibody identified immunoreactive signal bands in approximately 37\u201340, 43, 64, and 68\u2009kDa, consistent with CBD. Genetic analysis did not reveal any known pathogenic mutations in the microtubule\u2010associated protein tau gene (<jats:italic>MAPT<\/jats:italic>). Our case was characterized by the HCB sign and concordant neuropathological changes in the pons. CBD should be considered an underlying pathology of the HCB sign, even though the pontocerebellar changes would be unusual in CBD cases.<\/jats:p>","DOI":"10.1111\/neup.12745","type":"journal-article","created":{"date-parts":[[2021,7,28]],"date-time":"2021-07-28T23:57:55Z","timestamp":1627516675000},"page":"376-380","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["The hot cross bun sign in corticobasal degeneration"],"prefix":"10.1111","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7045-7098","authenticated-orcid":false,"given":"Takashi","family":"Ando","sequence":"first","affiliation":[{"name":"Department of Neurology Nagoya University Graduate School of Medicine  Nagoya Japan"},{"name":"Department of Neuropathology Institute for Medical Science of Aging, Aichi Medical University  Nagakute Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fuji","family":"Yokoi","sequence":"additional","affiliation":[{"name":"Department of Neurology Narita Memorial Hospital  Toyohashi Japan"},{"name":"Department of Neurology Atsumi Hospital  Tahara Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6493-8976","authenticated-orcid":false,"given":"Yuichi","family":"Riku","sequence":"additional","affiliation":[{"name":"Department of Neurology Nagoya University Graduate School of Medicine  Nagoya Japan"},{"name":"Department of Neuropathology Institute for Medical Science of Aging, Aichi Medical University  Nagakute Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4712-7562","authenticated-orcid":false,"given":"Akio","family":"Akagi","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Institute for Medical Science of Aging, Aichi Medical University  Nagakute Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroaki","family":"Miyahara","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Institute for Medical Science of Aging, Aichi Medical University  Nagakute Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7415-8159","authenticated-orcid":false,"given":"Masato","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Department of Brain and Neurosciences Tokyo Metropolitan Institute of Medical Science  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masahisa","family":"Katsuno","sequence":"additional","affiliation":[{"name":"Department of Neurology Nagoya University Graduate School of Medicine  Nagoya Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mari","family":"Yoshida","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Institute 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