FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3028402743> ?p ?o ?g. }

• W3028402743 endingPage "1571" @default.
• W3028402743 startingPage "1555" @default.
• W3028402743 abstract "The syndromes caused by frontotemporal lobar degeneration have highly heterogeneous and overlapping clinical features. There has been great progress in the refinement of clinical diagnostic criteria in the past decade, but we propose that a better understanding of aetiology, pathophysiology and symptomatic treatments can arise from a transdiagnostic approach to clinical phenotype and brain morphometry. In a cross-sectional epidemiological study, we examined 310 patients with a syndrome likely to be caused by frontotemporal lobar degeneration, including behavioural variant frontotemporal dementia, non-fluent, and semantic variants of primary progressive aphasia (PPA), progressive supranuclear palsy and corticobasal syndrome. We included patients with logopenic PPA and those who met criteria for PPA but not a specific subtype. To date, 49 patients have a neuropathological diagnosis. A principal component analysis identified symptom dimensions that broadly recapitulated the core features of the main clinical syndromes. However, the subject-specific scores on these dimensions showed considerable overlap across the diagnostic groups. Sixty-two per cent of participants had phenotypic features that met the diagnostic criteria for more than one syndrome. Behavioural disturbance was prevalent in all groups. Forty-four per cent of patients with corticobasal syndrome had progressive supranuclear palsy-like features and 30% of patients with progressive supranuclear palsy had corticobasal syndrome-like features. Many patients with progressive supranuclear palsy and corticobasal syndrome had language impairments consistent with non-fluent variant PPA while patients with behavioural variant frontotemporal dementia often had semantic impairments. Using multivariate source-based morphometry on a subset of patients (n = 133), we identified patterns of covarying brain atrophy that were represented across the diagnostic groups. Canonical correlation analysis of clinical and imaging components found three key brain-behaviour relationships, with a continuous spectrum across the cohort rather than discrete diagnostic entities. In the 46 patients with follow-up (mean 3.6 years) syndromic overlap increased with time. Together, these results show that syndromes associated with frontotemporal lobar degeneration do not form discrete mutually exclusive categories from their clinical features or structural brain changes, but instead exist in a multidimensional spectrum. Patients often manifest diagnostic features of multiple disorders while deficits in behaviour, movement and language domains are not confined to specific diagnostic groups. It is important to recognize individual differences in clinical phenotype, both for clinical management and to understand pathogenic mechanisms. We suggest that a transdiagnostic approach to the spectrum of frontotemporal lobar degeneration syndromes provides a useful framework with which to understand disease aetiology, progression, and heterogeneity and to target future treatments to a higher proportion of patients." @default.
• W3028402743 created "2020-05-29" @default.
• W3028402743 creator A5001733249 @default.
• W3028402743 creator A5012413697 @default.
• W3028402743 creator A5025003387 @default.
• W3028402743 creator A5031509032 @default.
• W3028402743 creator A5031686265 @default.
• W3028402743 creator A5038408775 @default.
• W3028402743 creator A5042708762 @default.
• W3028402743 creator A5048423063 @default.
• W3028402743 creator A5049135281 @default.
• W3028402743 creator A5075493660 @default.
• W3028402743 creator A5077445938 @default.
• W3028402743 creator A5080102075 @default.
• W3028402743 creator A5089532557 @default.
• W3028402743 date "2020-05-01" @default.
• W3028402743 modified "2023-10-17" @default.
• W3028402743 title "Redefining the multidimensional clinical phenotypes of frontotemporal lobar degeneration syndromes" @default.
• W3028402743 cites W1774563006 @default.
• W3028402743 cites W1862190875 @default.
• W3028402743 cites W1929164320 @default.
• W3028402743 cites W1980947267 @default.
• W3028402743 cites W1981844785 @default.
• W3028402743 cites W1996314668 @default.
• W3028402743 cites W1998433809 @default.
• W3028402743 cites W2010681077 @default.
• W3028402743 cites W2011349580 @default.
• W3028402743 cites W2017654994 @default.
• W3028402743 cites W2018735312 @default.
• W3028402743 cites W2018996065 @default.
• W3028402743 cites W2021422157 @default.
• W3028402743 cites W2021490774 @default.
• W3028402743 cites W2025077501 @default.
• W3028402743 cites W2055531721 @default.
• W3028402743 cites W2058161128 @default.
• W3028402743 cites W2064907795 @default.
• W3028402743 cites W2067439875 @default.
• W3028402743 cites W2070726861 @default.
• W3028402743 cites W2073704038 @default.
• W3028402743 cites W2073845553 @default.
• W3028402743 cites W2086220442 @default.
• W3028402743 cites W2090664364 @default.
• W3028402743 cites W2096609895 @default.
• W3028402743 cites W2102286051 @default.
• W3028402743 cites W2103717170 @default.
• W3028402743 cites W2106279576 @default.
• W3028402743 cites W2106786486 @default.
• W3028402743 cites W2106922104 @default.
• W3028402743 cites W2107099006 @default.
• W3028402743 cites W2112275614 @default.
• W3028402743 cites W2119720840 @default.
• W3028402743 cites W2122211082 @default.
• W3028402743 cites W2123692283 @default.
• W3028402743 cites W2124873909 @default.
• W3028402743 cites W2125077747 @default.
• W3028402743 cites W2128030851 @default.
• W3028402743 cites W2130156284 @default.
• W3028402743 cites W2137161718 @default.
• W3028402743 cites W2138190873 @default.
• W3028402743 cites W2142720569 @default.
• W3028402743 cites W2144707045 @default.
• W3028402743 cites W2146281101 @default.
• W3028402743 cites W2148465790 @default.
• W3028402743 cites W2151219135 @default.
• W3028402743 cites W2151427255 @default.
• W3028402743 cites W2152047920 @default.
• W3028402743 cites W2154633730 @default.
• W3028402743 cites W2158292481 @default.
• W3028402743 cites W2163975394 @default.
• W3028402743 cites W2168365826 @default.
• W3028402743 cites W2218579689 @default.
• W3028402743 cites W2272468257 @default.
• W3028402743 cites W2278265690 @default.
• W3028402743 cites W2303577120 @default.
• W3028402743 cites W2304803376 @default.
• W3028402743 cites W2334692654 @default.
• W3028402743 cites W2342759717 @default.
• W3028402743 cites W2343836310 @default.
• W3028402743 cites W2443158049 @default.
• W3028402743 cites W2472597773 @default.
• W3028402743 cites W2494252203 @default.
• W3028402743 cites W2531444270 @default.
• W3028402743 cites W2541769071 @default.
• W3028402743 cites W2551546166 @default.
• W3028402743 cites W2582092343 @default.
• W3028402743 cites W2582619151 @default.
• W3028402743 cites W2612001459 @default.
• W3028402743 cites W2618416220 @default.
• W3028402743 cites W2619097621 @default.
• W3028402743 cites W2623663856 @default.
• W3028402743 cites W2625115119 @default.
• W3028402743 cites W2625796101 @default.
• W3028402743 cites W2734670557 @default.
• W3028402743 cites W2742995083 @default.
• W3028402743 cites W2743089093 @default.
• W3028402743 cites W275504699 @default.
• W3028402743 cites W2760832975 @default.
• W3028402743 cites W2773684004 @default.

• next