Abstract

Vigilance complaints often occur in people with narcolepsy type 1 and severely impair effective daytime functioning. We tested the feasibility of a three-level sustained attention to response task (SART) paradigm within a magnetic resonance imaging (MRI) environment to understand brain architecture underlying vigilance regulation in individuals with narcolepsy type 1. Twelve medication-free people with narcolepsy type 1 and 11 matched controls were included. The SART included four repetitions of a baseline block and two difficulty levels requiring moderate and high vigilance. Outcome measures were between and within-group performance indices on error rates and reaction times, and functional MRI (fMRI) parameters: mean activity during the task and between-group activity differences across the three conditions and related to changes in activation over time (time-on-task) and error-related activity. Patients—but not controls—made significantly more mistakes with increasing difficulty. The modified SART is a feasible MRI vigilance task showing similar task-positive brain activity in both groups within the cingulo-opercular, frontoparietal, arousal, motor, and visual networks. During blocks of higher vigilance demand, patients had significantly lower activation in these regions than controls. Patients had lower error-related activity in the left pre- and postcentral gyrus. The time-on-task activity differences between groups suggest that those with narcolepsy are insufficiently capable of activating attention- and arousal-related regions when transitioning from attention initiation to stable attention, specifically when vigilance demand is high. They also show lower inhibitory motor activity in relation to errors, suggesting impaired executive functioning.

Highlights

  • Narcolepsy type 1 is a severely disabling neurological condition caused by a selective loss of hypocretin-producing neurons in the lateral and posterior hypothalamus [1,2]

  • We report on a functional magnetic resonance imaging (MRI) study investigating the neurobiological basis of the vigilance difficulties in people with narcolepsy type 1 using the sustained attention to response task (SART)

  • We suggest that maximal vigilance capacities have already been reached by people with narcolepsy type 1 in the early stage of the higher difficulty level, whereas controls are able to upregulate their cognitive effort to remain vigilant throughout the second half

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Summary

Introduction

Narcolepsy type 1 is a severely disabling neurological condition caused by a selective loss of hypocretin-producing neurons in the lateral and posterior hypothalamus [1,2]. It is characterized by excessive daytime sleepiness (EDS), cataplexy, sleep paralysis, hypnagogic hallucinations and disturbed nocturnal sleep. Brain Sci. 2020, 10, 419 vigilant is essential for effective daytime functioning [6,7]. We report on a functional MRI (fMRI) study investigating the neurobiological basis of the vigilance difficulties in people with narcolepsy type 1 using the sustained attention to response task (SART)

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