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- W2113823638 abstract "Frontotemporal lobar degeneration with ubiquitinated inclusions (FTLD-U) is the most common neuropathology associated with the clinical syndrome of frontotemporal dementia (FTD). Recently, TDP-43 was identified as the ubiquitinated pathological protein in both FTLD-U and sporadic amyotrophic lateral sclerosis. Although a number of studies have now confirmed that most sporadic and familial cases of FTLD-U are TDP-43 proteinopathies, there are exceptions. All cases within our neuropathology database, that had previously been given a pathological diagnosis of FTLD-U, were re-evaluated using TDP-43 immunohistochemistry. Cases with FTLD-U but no abnormal TDP-43 immunostaining were referred to as “atypical” FTLD-U (aFTLD-U) and underwent further clinical and pathological characterization. Six cases with aFTLD-U pathology were identified. All were sporadic and had very early onset FTD (mean age = 35 years), characterized by severe progressive psychobehavioural abnormalities in the absence of significant aphasia, cognitive-intellectual dysfunction or motor features. The neuropathological features were highly consistent, with small, round, neuronal cytoplasmic inclusions that were immunoreactive for ubiquitin (ub-ir), but negative for tau, α-synuclein, intermediate filaments and TDP-43. Cytoplasmic inclusions were most numerous in the neocortex, dentate granule cells and hippocampal pyramidal neurons. Ub-ir neuronal intranuclear inclusions were also present in neocortical and hippocampal neurons and had the unusual appearance of straight, curved, or twisted filaments. We believe that these cases represent a new entity that is clinically and pathologically distinct from all currently recognized subtypes of FTLD. Moreover, the existence of such cases indicates that the designations of “FTLD-U” and “TDP-43 proteinopathy” should not be considered to be synonymous." @default.
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- W2113823638 date "2008-07-01" @default.
- W2113823638 modified "2023-09-27" @default.
- W2113823638 title "P2-188: Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusions" @default.
- W2113823638 doi "https://doi.org/10.1016/j.jalz.2008.05.1262" @default.
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