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.

No abstract

Mutations were identified in nine COL1A1/COL1A2 associated with OI type I-IV genes by scanning with DHPLC. Software was used to detect point mutation and large deletions/insertions in COL1A1 and COL1A2 genes.

Background Baloxavir marboxil (baloxavir), an anti-influenza drug with a novel mechanism of action, is expected to reduce influenza transmission by rapid reduction of viral load. The incidence of household transmission was compared between index patients (IPs) treated with baloxavir and those with neuraminidase inhibitors. Methods Using a Japanese claims database provided by JMDC Inc., the first family members with influenza diagnosis during 2018/2019 influenza season were identified as IPs, and the diagnosis date was designated Day 1. According to the anti-influenza drug dispensed to the IP, their families were classified into oral baloxavir group and three controls; oral oseltamivir group (a primary control), inhaled zanamivir group, and inhaled laninamivir group. A household transmission was defined as influenza diagnosed for any non-IP family members during Days 3−8. The incidence of household transmission was compared between groups using a logistic regression model adjusting backgrounds of IPs. Results The proportion of families with household transmission was 17.98% (15,226/84,672) in the baloxavir group and 24.16% (14,983/62,004) in the oseltamivir group. The covariate-adjusted odds ratio (oseltamivir/baloxavir) was 1.09 [95% confidence interval, 1.05–1.12], which indicated significantly lower incidence in the baloxavir group. Adjusted odds ratios (controls/baloxavir) against zanamivir and laninamivir were 0.93 [0.89–0.97] and 0.99 [0.96–1.02], respectively. Conclusions Baloxavir may contribute to reduce the incidence of household transmission compared with oseltamivir, which is widely used for oral influenza treatment. In comparison between baloxavir and inhalants, a similar reduction was not shown and it might be due to unmeasured confounding by administration route differences.