2015
DOI: 10.1093/brain/awv220
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TDP-43 proteinopathies: pathological identification of brain regions differentiating clinical phenotypes

Abstract: The pathological sequestration of TAR DNA-binding protein 43 (TDP-43, encoded by TARDBP) into cytoplasmic pathological inclusions characterizes the distinct clinical syndromes of amyotrophic lateral sclerosis and behavioural variant frontotemporal dementia, while also co-occurring in a proportion of patients with Alzheimer's disease, suggesting that the regional concentration of TDP-43 pathology has most relevance to specific clinical phenotypes. This has been reflected in the three different pathological stag… Show more

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Cited by 98 publications
(88 citation statements)
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“…Family history for ALS was more prevalent in pure bvFTD cases with TDP-43 in the hypoglossal nucleus, but this did not reach significance compared to other bvFTD cohorts (p = 0.06). The recent topographical staging of TDP-43 pathology in bvFTD and ALS [6,7] demonstrated that TDP-43 initiated in the motor system network in ALS is also seen in the majority of bvFTD cases, despite the absence of ALS in over 50% of these [6,21]. In a recent analysis [21], we identified the hypoglossal nucleus as a key brain region in discriminating between cases with and without ALS and the present study corroborates this finding in bvFTD cases.…”
Section: Demographic Featuressupporting
confidence: 88%
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“…Family history for ALS was more prevalent in pure bvFTD cases with TDP-43 in the hypoglossal nucleus, but this did not reach significance compared to other bvFTD cohorts (p = 0.06). The recent topographical staging of TDP-43 pathology in bvFTD and ALS [6,7] demonstrated that TDP-43 initiated in the motor system network in ALS is also seen in the majority of bvFTD cases, despite the absence of ALS in over 50% of these [6,21]. In a recent analysis [21], we identified the hypoglossal nucleus as a key brain region in discriminating between cases with and without ALS and the present study corroborates this finding in bvFTD cases.…”
Section: Demographic Featuressupporting
confidence: 88%
“…The recent topographical staging of TDP-43 pathology in bvFTD and ALS [6,7] demonstrated that TDP-43 initiated in the motor system network in ALS is also seen in the majority of bvFTD cases, despite the absence of ALS in over 50% of these [6,21]. In a recent analysis [21], we identified the hypoglossal nucleus as a key brain region in discriminating between cases with and without ALS and the present study corroborates this finding in bvFTD cases. Further support for the segregation of bvFTD cases by TDP-43 inclusions in the hypoglossal nucleus was evidenced here by differences in disease duration and postmortem brain weights, with 'intermediate' levels of atrophy in bvFTD cases with end-stage ALS (Figure 2).…”
Section: Demographic Featuressupporting
confidence: 88%
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