title: Incipient intranuclear inclusion body disease in a 78-year-old woman.
abstract: We report an incipient case of intranuclear inclusion body disease (INIBD) in a 78-year-old woman. No apparent neurological symptoms were noticed during the clinical course. Post mortem examination revealed widespread occurrence of eosinophilic intranuclear inclusions in neuronal and glial cells of the central and peripheral nervous systems, as well as in parenchymal cells of the visceral organs. The inclusions were observed more frequently in glial cells than in neuronal cells. Ultrastructurally, the inclusions consisted of granular and filamentous material. Immunohistochemically, the inclusions were positive for ubiquitin, ubiquitin-related proteins (NEDD8 ultimate buster 1, small ubiquitin modifier-1, small ubiquitin modifier-2 and p62), promyelocytic leukemia protein and abnormally expanded polyglutamine. Consistent with previous studies, the vast majority of inclusion-bearing glial cells were astrocytes. Furthermore, p25α-positive oligodendrocytes rarely contained intranuclear inclusions. These findings suggest that INIBD may occur in non-demented elderly individuals and that oligodendrocyte is also involved in the disease process of INIBD.
authors.affiliation: Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki, Japan. neuropal@cc.hirosaki-u.ac.jp
authors.forename: Fumiaki
authors.initials: F
authors.lastname: Mori
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Miki
authors.forename: Kunikazu
authors.initials: K
authors.lastname: Tanji
authors.forename: Eriko
authors.initials: E
authors.lastname: Ogura
authors.forename: Norito
authors.initials: N
authors.lastname: Yagihashi
authors.forename: Poul H
authors.initials: PH
authors.lastname: Jensen
authors.forename: Koichi
authors.initials: K
authors.lastname: Wakabayashi
mesh_terms: D000368:Aged; D001253:Astrocytes / Q000473:pathology; D001927:Brain Diseases / Q000473:pathology*; D003920:Diabetes Mellitus; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D045586:Intranuclear Inclusion Bodies / Q000648:ultrastructure*; D046529:Microscopy, Electron, Transmission; D019636:Neurodegenerative Diseases / Q000473:pathology*; D009836:Oligodendroglia / Q000473:pathology