{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,29]],"date-time":"2026-06-29T08:33:22Z","timestamp":1782722002464,"version":"3.54.5"},"reference-count":19,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,1,10]],"date-time":"2020-01-10T00:00:00Z","timestamp":1578614400000},"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","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":[[2020,6]]},"abstract":"<jats:p>Corticobasal degeneration (CBD) is a rare progressive neurodegenerative disorder characterized by asymmetric presentation of cerebral cortex signs, cortical sensory disturbance and extrapyramidal signs. Herein, we report a case of a 66\u2010year\u2010old Japanese woman who presented with apraxia of the right hand. She subsequently developed postural instability and cognitive impairments that rapidly worsened. One and a half years later, the patient was wheelchair\u2010bound and severely demented. Brain magnetic resonance imaging revealed left dominant atrophy of the frontoparietal lobe. There was a hyperintense lesion in the deep white matter expanding toward the subcortical area on fluid\u2010attenuated inversion recovery (FLAIR) images. In order to rule out the possibility of an intracranial tumor such as an astrocytoma or malignant lymphoma, we performed a brain biopsy of the left frontal middle gyrus. The patient became bedridden and showed akinetic mutism 1\u2009year after biopsy. Pathological examination revealed a large amount of 4\u2010repeat tau\u2010immunoreactive neuropil threads scattered predominantly in the corticomedullary junction and tau\u2010immunoreactive structures, consistent with CBD. Immunostaining for p53 showed no positive cells, and there were very few Ki\u201067\u2010positive cells. On immunoblots of sarkosyl\u2010insoluble brain extracts, a major doublet of 64 and 68\u2009kDa full\u2010length tau with two closely related fragments of approximately 37\u2009kDa were detected. Based on these results, the patient was pathologically diagnosed as having CBD, excluding the possibility of tumor. Taken together with previous similar case reports, our findings indicate that a deep white matter hyperintense lesion on FLAIR images may be a useful clue to CBD, predicting rapid clinical progression with severe dementia based on severe white matter degeneration with a large amount of tau accumulation on pathological examination.<\/jats:p>","DOI":"10.1111\/neup.12638","type":"journal-article","created":{"date-parts":[[2020,1,11]],"date-time":"2020-01-11T05:00:31Z","timestamp":1578718831000},"page":"287-294","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Corticobasal degeneration with deep white matter lesion diagnosed by brain biopsy"],"prefix":"10.1111","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9336-1168","authenticated-orcid":false,"given":"Akira","family":"Arakawa","sequence":"first","affiliation":[{"name":"Department of Neurology Tokyo Teishin Hospital  Tokyo 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