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• W1935627301 abstract "Phosphorylated TDP-43 (pTDP-43) is the pathological protein responsible for amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease. Recently, it has been reported that accumulation of pTDP-43 can occur in the brains of patients with argyrophilic grain disease (AGD), in which phosphorylated 4-repeat tau is the pathological protein. To elucidate the association of ALS with AGD, we examined the brains from 37 consecutively autopsied patients with sporadic ALS (age range 45-84 years, mean 71.5 ± 9.0 years).Sections from the frontotemporal lobe were stained with the Gallyas-Braak method and also immunostained with antibodies against phosphorylated tau, 4-repeat tau and pTDP-43.Fourteen (38%) of the 37 ALS patients were found to have AGD. With regard to staging, 5 of these 14 cases were rated as I, 4 as II and 5 as III. pTDP-43 immunohistochemistry revealed the presence of positive neuronal and glial cytoplasmic inclusions in the affected medial temporal lobe in many cases (93% and 64%, respectively). On the other hand, pTDP-43-positive small structures corresponding to argyrophilic grains were observed only in one case. A significant correlation was found between AGD and the Braak stage for neurofibrillary pathology (stage range 0-V, mean 2.1). However, there were no significant correlations between AGD and any other clinicopathological features, including dementia.The present findings suggest that co-occurrence of AGD in ALS is not uncommon, and in fact comparable with that in a number of diseases belonging to the tauopathies or α-synucleinopathies." @default.
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• W1935627301 date "2012-01-06" @default.
• W1935627301 modified "2023-09-24" @default.
• W1935627301 title "Co-occurrence of argyrophilic grain disease in sporadic amyotrophic lateral sclerosis" @default.
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• W1935627301 doi "https://doi.org/10.1111/j.1365-2990.2011.01175.x" @default.
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