Cognitive phenotypes of sequential staging in amyotrophic lateral sclerosis

Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
Dorothée LuléAlbert C Ludolph

Abstract

Sequential spread of TDP-43 load in the brain may be a pathological characteristic of amyotrophic lateral sclerosis (ALS). Diffusion tensor imaging (DTI) is a magnetic resonance imaging (MRI) based marker of this pathological feature. Cognitive deficits known to be present in a subset of ALS patients might act as an additional in vivo clinical marker of disease spread. N = 139 patients with ALS were tested with the Edinburgh Cognitive and Behavioural ALS screen (ECAS) in addition to DTI brain measures of pathological spread. Executive function, memory and disinhibited behaviour were selected for Cognitive-Staging criteria, as these cognitive functions are attributed to cerebral areas analogous to the pattern of MRI markers of TDP-43 pathology. ROC curve analyses were performed to define cut-off scores for cognitive stages 2 (executive function), stage 3 (disinhibited behaviour) and stage 4 (memory), and staging was performed according to the cognitive profile subsequently. Associations of Cognitive-Staging (stage 2-4) and MRI-Staging measures were determined. In total, 77 patients (55%) performed below ROC cut-off scores in either executive function or memory or both and/or were reported to have disinhibited behaviour which per...Continue Reading

Citations

Jul 18, 2018·Annals of Neurology·Thiago Junqueira Ribeiro RezendeMarcondes C França
Sep 16, 2018·Journal of Neurology·Morten MagelssenReidun Førde
Feb 19, 2019·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Rubika BalendraAmmar Al-Chalabi
Dec 13, 2018·The Journal of Neuropsychiatry and Clinical Neurosciences·Aldrin Pedroza MartinsLeonardo Cruz de Souza
Jan 16, 2020·Expert Review of Neurotherapeutics·Jan Kassubek, Hans-Peter Müller
Nov 2, 2019·Journal of Neurology, Neurosurgery, and Psychiatry·Dorothée E Lulé, Albert C Ludolph
Mar 12, 2020·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Isabel K GosseltMichael A Van Es
Apr 28, 2020·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Biying YangXiaolei Shi
Jun 3, 2020·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Enrica BersanoLetizia Mazzini
May 18, 2020·Neurology·Hannelore K van der BurghLeonard H van den Berg
Aug 29, 2020·Journal of Neurology, Neurosurgery, and Psychiatry·Dorothée E LuléAlbert C Ludolph
Aug 7, 2019·Neuropathology and Applied Neurobiology·J M GregoryC Smith
Oct 16, 2018·Frontiers in Neuroscience·Hans-Peter Müller, Jan Kassubek
Nov 28, 2018·Annals of Clinical and Translational Neurology·Abdullah IshaqueSanjay Kalra
Dec 14, 2018·Dementia & Neuropsychologia·Ratko RadakovicMichael Hornberger
Dec 4, 2020·EMBO Molecular Medicine·Katerina PlacekCorey T McMillan
Aug 20, 2020·Cortex; a Journal Devoted to the Study of the Nervous System and Behavior·Monica ConsonniGiuseppe Lauria
Mar 13, 2021·Journal of Neurology·Anna BehlerJan Kassubek
Mar 28, 2021·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Fabiola De MarchiUNKNOWN SIgN
Apr 6, 2021·Therapeutic Advances in Chronic Disease·Hans-Peter MüllerJan Kassubek
Jan 5, 2019·Revue neurologique·P CorciaP Couratier
Nov 7, 2020·Brain Imaging and Behavior·Sneha ChenjiUNKNOWN Canadian ALS Neuroimaging Consortium (CALSNIC)
Jan 7, 2022·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·Alessio MaranzanoNicola Ticozzi

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