{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T10:25:34Z","timestamp":1785925534046,"version":"3.56.0"},"reference-count":24,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,12,26]],"date-time":"2021-12-26T00:00:00Z","timestamp":1640476800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2022,2]]},"abstract":"<jats:p>Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease characterized by appearance of eosinophilic hyaline intranuclear inclusions. While the main symptoms of adult\u2010onset NIID are dementia or limb weakness, some patients present with encephalitic episodes and transient neurological symptoms. The pathophysiology of these acute, transient symptoms, however, remains unknown. Here, we describe an autopsy case of adult\u2010onset NIID with progressive dementia and transient hemiparesis. The patient was a 70\u2010year\u2010old man without a relevant family history, and initially presented with progressive dementia. He then exhibited transient left hemiparesis at 75\u2009years of age and died of ureteral cancer at 77\u2009years of age. Neuropathological examination revealed the presence of multiple areas of focal spongiosis in the subcortical white matter and patchy myelin pallor of the white matter, as in previous reports. However, perivascular areas were preserved even in the damaged white matter. In addition, dense glial fibrillary acidic protein (GFAP)\u2010immunoreactive astrocytic processes were observed in these areas. [Correction added on 23 January 2022, after first online publication: the preceding sentence has been corrected to improve readability.] GFAP immunohistochemistry revealed decreased density and morphological abnormalities of astrocytes in the affected white matter. These pathological findings might reflect blood\u2013brain barrier impairment and dysregulation of blood flow, which may be related to the pathophysiology of the acute, transient symptoms observed in NIID.<\/jats:p>","DOI":"10.1111\/neup.12778","type":"journal-article","created":{"date-parts":[[2021,12,27]],"date-time":"2021-12-27T00:02:15Z","timestamp":1640563335000},"page":"66-73","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["An autopsy case of adult\u2010onset neuronal intranuclear inclusion disease with perivascular preservation in cerebral white matter"],"prefix":"10.1111","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9817-3337","authenticated-orcid":false,"given":"Daisuke","family":"Yoshii","sequence":"first","affiliation":[{"name":"Department of Neurology Kyoto University Graduate School of Medicine  Kyoto 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