Bocchetta, M;
Iglesias Espinosa, MDM;
Lashley, T;
Warren, JD;
Rohrer, JD;
(2020)
In vivo staging of frontotemporal lobar degeneration TDP-43 type C pathology.
Alzheimer's Research & Therapy
, 12
(1)
, Article 34. 10.1186/s13195-020-00600-x.
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Abstract
Background: TDP-43 type C is one of the pathological forms of frontotemporal lobar degeneration (FTLD) and mainly associated clinically with the semantic variant of primary progressive aphasia (svPPA). We aimed to define in vivo the sequential pattern of neuroanatomical involvement in a cohort of patients with FTLD-TDP type C pathology. Methods: We extracted the volumes of a set of cortical and subcortical regions from MRI scans of 19 patients with post mortem confirmed TDP-43 type C pathology (all with left hemisphere-predominant atrophy at baseline). In the initial development phase, we used w-scores computed from 81 cognitively normal controls to define a set of sequential stages of neuroanatomical involvement within the FTLD-TDP type C cohort where a w-score of < − 1.65 was considered abnormal. In a subsequent validation phase, we used 31 follow-up scans from 14 of the 19 patients in the same cohort to confirm the staging model. Results: Four sequential stages were identified in the initial development phase. Stage 1 was defined by atrophy in the left amygdala, medial temporal cortex, temporal pole, lateral temporal cortex and right medial temporal cortex; Stage 2 by atrophy in the left supratemporal cortex; Stage 3 by atrophy in the right anterior insula; and Stage 4 by atrophy in the right accumbens. In the validation phase, calculation of w-scores in the longitudinal scans confirmed the staging system, with all patients either staying in the same stage or progressing to a later stage at follow-up. Conclusion: In vivo imaging is able to detect distinct stages of neuroanatomical involvement in FTLD-TDP type C pathology. Using an imaging-derived staging system allows a more refined stratification of patients with svPPA during life.
| Type: | Article |
|---|---|
| Title: | In vivo staging of frontotemporal lobar degeneration TDP-43 type C pathology |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1186/s13195-020-00600-x |
| Publisher version: | http://dx.doi.org/10.1186/s13195-020-00600-x |
| Language: | English |
| Additional information: | © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/10094242 |
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