| pubmed-article:21908872 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:21908872 | lifeskim:mentions | umls-concept:C0040300 | lld:lifeskim |
| pubmed-article:21908872 | lifeskim:mentions | umls-concept:C0030664 | lld:lifeskim |
| pubmed-article:21908872 | lifeskim:mentions | umls-concept:C0751072 | lld:lifeskim |
| pubmed-article:21908872 | lifeskim:mentions | umls-concept:C1519316 | lld:lifeskim |
| pubmed-article:21908872 | lifeskim:mentions | umls-concept:C0205210 | lld:lifeskim |
| pubmed-article:21908872 | pubmed:issue | Pt 9 | lld:pubmed |
| pubmed-article:21908872 | pubmed:dateCreated | 2011-9-12 | lld:pubmed |
| pubmed-article:21908872 | pubmed:abstractText | Relating clinical symptoms to neuroanatomical profiles of brain damage and ultimately to tissue pathology is a key challenge in the field of neurodegenerative disease and particularly relevant to the heterogeneous disorders that comprise the frontotemporal lobar degeneration spectrum. Here we present a retrospective analysis of clinical, neuropsychological and neuroimaging (volumetric and voxel-based morphometric) features in a pathologically ascertained cohort of 95 cases of frontotemporal lobar degeneration classified according to contemporary neuropathological criteria. Forty-eight cases (51%) had TDP-43 pathology, 42 (44%) had tau pathology and five (5%) had fused-in-sarcoma pathology. Certain relatively specific clinicopathological associations were identified. Semantic dementia was predominantly associated with TDP-43 type C pathology; frontotemporal dementia and motoneuron disease with TDP-43 type B pathology; young-onset behavioural variant frontotemporal dementia with FUS pathology; and the progressive supranuclear palsy syndrome with progressive supranuclear palsy pathology. Progressive non-fluent aphasia was most commonly associated with tau pathology. However, the most common clinical syndrome (behavioural variant frontotemporal dementia) was pathologically heterogeneous; while pathologically proven Pick's disease and corticobasal degeneration were clinically heterogeneous, and TDP-43 type A pathology was associated with similar clinical features in cases with and without progranulin mutations. Volumetric magnetic resonance imaging, voxel-based morphometry and cluster analyses of the pathological groups here suggested a neuroanatomical framework underpinning this clinical and pathological diversity. Frontotemporal lobar degeneration-associated pathologies segregated based on their cerebral atrophy profiles, according to the following scheme: asymmetric, relatively localized (predominantly temporal lobe) atrophy (TDP-43 type C); relatively symmetric, relatively localized (predominantly temporal lobe) atrophy (microtubule-associated protein tau mutations); strongly asymmetric, distributed atrophy (Pick's disease); relatively symmetric, predominantly extratemporal atrophy (corticobasal degeneration, fused-in-sarcoma pathology). TDP-43 type A pathology was associated with substantial individual variation; however, within this group progranulin mutations were associated with strongly asymmetric, distributed hemispheric atrophy. We interpret the findings in terms of emerging network models of neurodegenerative disease: the neuroanatomical specificity of particular frontotemporal lobar degeneration pathologies may depend on an interaction of disease-specific and network-specific factors. | lld:pubmed |
| pubmed-article:21908872 | pubmed:grant | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:21908872 | pubmed:commentsCorrections | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:21908872 | pubmed:language | eng | lld:pubmed |
| pubmed-article:21908872 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:21908872 | pubmed:citationSubset | AIM | lld:pubmed |
| pubmed-article:21908872 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:21908872 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:21908872 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:21908872 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:21908872 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:21908872 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:21908872 | pubmed:month | Sep | lld:pubmed |
| pubmed-article:21908872 | pubmed:issn | 1460-2156 | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:BorroniBarbar... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:HardyJohnJ | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:WarrenJason... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:HoltonJanice... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:LashleyTammar... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:ReveszTamasT | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:SchottJonatha... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:RossorMartin... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:FoxNick CNC | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:KingAndrewA | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:de... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:IsaacsAdrian... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:Al-SarrajSafa... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:MeadSimonS | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:TroakesClaire... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:WarrenJane... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:BeckJonathanJ | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:RohrerJonatha... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:OurselinSebas... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:ClarksonMatth... | lld:pubmed |
| pubmed-article:21908872 | pubmed:author | pubmed-author:WarringtonEli... | lld:pubmed |
| pubmed-article:21908872 | pubmed:issnType | Electronic | lld:pubmed |
| pubmed-article:21908872 | pubmed:volume | 134 | lld:pubmed |
| pubmed-article:21908872 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:21908872 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:21908872 | pubmed:pagination | 2565-81 | lld:pubmed |
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| pubmed-article:21908872 | pubmed:meshHeading | pubmed-meshheading:21908872... | lld:pubmed |
| pubmed-article:21908872 | pubmed:meshHeading | pubmed-meshheading:21908872... | lld:pubmed |
| pubmed-article:21908872 | pubmed:meshHeading | pubmed-meshheading:21908872... | lld:pubmed |
| pubmed-article:21908872 | pubmed:meshHeading | pubmed-meshheading:21908872... | lld:pubmed |
| pubmed-article:21908872 | pubmed:meshHeading | pubmed-meshheading:21908872... | lld:pubmed |
| pubmed-article:21908872 | pubmed:year | 2011 | lld:pubmed |
| pubmed-article:21908872 | pubmed:articleTitle | Clinical and neuroanatomical signatures of tissue pathology in frontotemporal lobar degeneration. | lld:pubmed |
| pubmed-article:21908872 | pubmed:affiliation | Dementia Research Centre, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK. | lld:pubmed |
| pubmed-article:21908872 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:21908872 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| entrez-gene:317385 | entrezgene:pubmed | pubmed-article:21908872 | lld:entrezgene |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:21908872 | lld:pubmed |
