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International audience; Developmental and epileptic encephalopathies (DEE) are a group of neurodevelopmental disorders characterized by epileptic seizures associated with developmental delay or regression. DEE are genetically heterogeneous, and the proteins involved play roles in multiple pathways such as synaptic transmission, metabolism, neuronal development or maturation, transcriptional regulation, and intracellular trafficking. We performed whole exome sequencing on a consanguineous family with three children presenting an early onset (

International audience; Motor preparation, based on one's goals and expectations, allows for prompt reactions to stimulations from the environment. Proactive and reactive inhibitory mechanisms modulate this preparation and interact to allow a flexible control of responses. In this study, we investigate these two control mechanisms with an ad hoc cued Go/NoGo Simon paradigm in a within-subjects design, and by measuring subliminal motor activities through electromyographic recordings. Go cues instructed participants to prepare a response and wait for target onset to execute it (Go target) or inhibit it (NoGo target). Proactive inhibition keeps the prepared response in check, hence preventing false alarms. Preparing the cue-coherent effector in advance speeded up responses, even when it turned out to be the incorrect effector and reactive inhibition was needed to perform the action with the contralateral one. These results suggest that informative cues allow for the investigation of the interaction between proactive and reactive action inhibition. Partial errors' analysis suggests that their appearance in compatible conflict-free trials depends on cue type and prior preparatory motor activity. Motor preparation plays a key role in determining whether proactive inhibition is needed to flexibly control behavior, and it should be considered when investigating proactive/reactive inhibition.

Decision-making models based on evidence accumulation processes (the most prolific one being the drift-diffusion model – DDM) are widely used to draw inferences about latent psychological processes from chronometric data. While the observed goodness of fit in a wide range of tasks supports the model’s validity, the derived interpretations have yet to be sufficiently cross-validated with other measures that also reflect cognitive processing. To do so, we recorded electromyographic (EMG) activity along with response times (RT), and used it to decompose every RT into two components: a pre-motor (PMT) and motor time (MT). These measures were mapped to the DDM's parameters, thus allowing a test, beyond quality of fit, of the validity of the model’s assumptions and their usual interpretation. In two perceptual decision tasks, performed within a canonical task setting, we manipulated stimulus contrast, speed-accuracy trade-off, and response force, and assessed their effects on PMT, MT, and RT. Contrary to common assumptions, these three factors consistently affected MT. DDM parameter estimates of non-decision processes are thought to include motor execution processes, and they were globally linked to the recorded response execution MT. However, when the assumption of independence between decision and non-decision processes was not met, in the fastest trials, the link was weaker. Overall, the results show a fair concordance between model-based and EMG-based decompositions of RTs, but also establish some limits on the interpretability of decision model parameters linked to response execution.