| How predictive are temporal lobe changes of underlying TDP-43 pathology in the ALS-FTD continuum?
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| Ana Paula Arantes Bueno1, Maxime Bertoux2,3, Leonardo Cruz de Souza4, and Michael Hornberger2,3 |
1Center of Mathematics, Computation and Cognition, Universidade Federal do ABC, Santo André, Brazil
2Department of Medicine, Norwich Medical School, University of East Anglia, Norwich, United Kingdom
3Dementia and Complexity in Later Life, NHS Norfolk and Suffolk Foundation Trust, Norwich, United Kingdom
4Departament of Internal Medicine, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil |
| Corresponding Author:
Michael Hornberger ,Tel: +441603597139 , Fax: +441603593752 , Email: m.hornberger@uea.ac.uk |
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Received December 7, 2016 Revised: March 5, 2017 Accepted April 4, 2017 |
| Copyright © 2017 The Korean Society of Clinical Neurophysiology |
| This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium,
provided the original work is properly cited. |
| ABSTRACT |
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Detection of underling proteinopathies is becoming increasingly important across neurodegenerative conditions due to upcoming disease intervention trials. In this review, we explored how temporal lobe changes in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) can potentially predict underlying TDP-43 pathology subtypes in FTD. To date, emphasis has been given to frontal lobe changes in the study of the cognitive and behavioural impairments in both syndromes but an increasing number of pathological, imaging and neuropsychological studies suggest how temporal lobe changes could critically affect the cognition and behaviour of these conditions. In this current article, we reviewed pathological, imaging as well as clinical/neuropsychological findings of temporal involvement in the ALS-FTD continuum, how they relate to temporal lobe changes and the underlying TDP-43 pathology in FTD. Findings across studies show that TDP-43 pathology occurs and coincides in many structures in ALS and FTD, but especially in the temporal lobes. In particular, anterior and medial temporal lobes atrophy is consistently found in ALS and FTD. In addition, memory and language impairment as well as emotional and Theory of Mind processing deficits that are characteristics of the two diseases are highly correlated to temporal lobe dysfunction. We conclude by showing that temporal lobe changes due to TDP-43 type B might be particular predictive of TDP-43 type B pathology in behavioural variant FTD, which clearly needs to be investigated further in the future. |
| Key words:
Amyotrophic lateral sclerosis; Frontotemporal dementia; Memory; Temporal lobes |
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