guest ::
| Home > Articles > Published articles > Cognitive decline in amyotrophic lateral sclerosis : |
| Home > Articles > Published articles > Cognitive decline in amyotrophic lateral sclerosis : |
(Universitat de Barcelona) (Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas) (Institut d'Investigacions Biomèdiques August Pi i Sunyer) (Hospital del Mar (Barcelona, Catalunya)) (Universitat Autònoma de Barcelona) (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol) (Hospital Universitari MútuaTerrassa (Terrassa, Catalunya)) (Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas) (Universitat de Barcelona) (Universitat de Barcelona) (Universitat de Barcelona) (Institut d'Investigacions Biomèdiques August Pi i Sunyer) (Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas) (Medical University of Vienna)
| Date: | 2021 |
| Abstract: | Cognitive impairment and behavioral changes in amyotrophic lateral sclerosis (ALS) are now recognized as part of the disease. Whether it is solely related to the extent of TDP-43 pathology is currently unclear. We aim to evaluate the influence of age, genetics, neuropathological features, and concomitant pathologies on cognitive impairment in ALS patients. We analyzed a postmortem series of 104 ALS patients and retrospectively reviewed clinical and neuropathological data. We assessed the burden and extent of concomitant pathologies, the role of APOE ε4 and mutations, and correlated these findings with cognitive status. We performed a logistic regression model to identify which pathologies are related to cognitive impairment. Cognitive decline was recorded in 38. 5% of the subjects. Neuropathological features of frontotemporal lobar degeneration (FTLD) were found in 32. 7%, explaining most, but not all, cases with cognitive impairment. Extent of TDP-43 pathology and the presence of hippocampal sclerosis were associated with cognitive impairment. Mutation carriers presented a higher burden of TDP-43 pathology and FTLD more frequently than sporadic cases. Most cases (89. 4%) presented some degree of concomitant pathologies. The presence of concomitant pathologies was associated with older age at death. FTLD, but also Alzheimer's disease, were the predominant underlying pathologies explaining the cognitive impairment in ALS patients. In sum, FTLD explained the presence of cognitive decline in most but not all ALS cases, while other non-FTLD related findings can influence the cognitive status, particularly in older age groups. |
| Grants: | Ministerio de Economía, Industria y Competitividad PI16/01673 Instituto de Salud Carlos III PI19/00593 Fundació la Marató de TV3 20141610 Fundació la Marató de TV3 20143810 Fundació la Marató de TV3 20143710 Ministerio de Economía, Industria y Competitividad PI15/01618 Generalitat de Catalunya. Departament de Salut SLT002/16/00329 |
| Rights: | Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, i la comunicació pública de l'obra, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original. No es permet la creació d'obres derivades.  |
| Language: | Anglès |
| Document: | Article ; recerca ; Versió publicada |
| Subject: | Alzheimer's disease ; Amyotrophic lateral sclerosis ; ALS-FTD ; Frontotemporal dementia ; Neuropathology ; TDP-43 protein |
| Published in: | Brain Pathology, Vol. 31 (february 2021) , ISSN 1750-3639 |
