title: Executive Dysfunctions and Event-Related Brain Potentials in Patients with Amyotrophic Lateral Sclerosis.
abstract: A growing body of evidence implies psychological disturbances in amyotrophic lateral sclerosis (ALS). Specifically, executive dysfunctions occur in up to 50% of ALS patients. The recently shown presence of cytoplasmic aggregates (TDP-43) in ALS patients and in patients with behavioral variants of frontotemporal dementia suggests that these two disease entities form the extremes of a spectrum. The present study aimed at investigating behavioral and electrophysiological indices of conflict processing in patients with ALS. A non-verbal variant of the flanker task demanded two-choice responses to target stimuli that were surrounded by flanker stimuli which either primed the correct response or the alternative response (the latter case representing the conflict situation). Behavioral performance, event-related potentials (ERP), and lateralized readiness potentials (LRP) were analyzed in 21 ALS patients and 20 controls. In addition, relations between these measures and executive dysfunctions were examined. ALS patients performed the flanker task normally, indicating preserved conflict processing. In similar vein, ERP and LRP indices of conflict processing did not differ between groups. However, ALS patients showed enhanced posterior negative ERP waveform deflections, possibly indicating increased modulation of visual processing by frontoparietal networks in ALS. We also found that the presence of executive dysfunctions was associated with more error-prone behavior and enhanced LRP amplitudes in ALS patients, pointing to a prefrontal pathogenesis of executive dysfunctions and to a potential link between prefrontal and motor cortical functional dysregulation in ALS, respectively.
authors.affiliation: Department of Neurology, Hannover Medical School Hannover, Germany.
authors.forename: Caroline
authors.initials: C
authors.lastname: Seer
authors.affiliation: Department of Neurology, Hannover Medical School Hannover, Germany.
authors.forename: Stefanie
authors.initials: S
authors.lastname: Fürkötter
authors.affiliation: Department of Neurology, Hannover Medical School Hannover, Germany.
authors.forename: Maj-Britt
authors.initials: MB
authors.lastname: Vogts
authors.affiliation: Department of Neurology, Hannover Medical School Hannover, Germany.
authors.forename: Florian
authors.initials: F
authors.lastname: Lange
authors.affiliation: Department of Neurology, Hannover Medical SchoolHannover, Germany; Department of Neurology, Otto-von-Guericke University MagdeburgMagdeburg, Germany; Department of Neurology, German Center for Neurodegenerative DiseasesMagdeburg, Germany.
authors.forename: Susanne
authors.initials: S
authors.lastname: Abdulla
authors.affiliation: Department of Neurology, Hannover Medical School Hannover, Germany.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Dengler
authors.affiliation: Department of Neurology, Hannover Medical School Hannover, Germany.
authors.forename: Susanne
authors.initials: S
authors.lastname: Petri
authors.affiliation: Department of Neurology, Hannover Medical School Hannover, Germany.
authors.forename: Bruno
authors.initials: B
authors.lastname: Kopp
keywords: ERP; LRP; amyotrophic lateral sclerosis; attention; executive functions