Abstract

Background: Cognitive dysfunction is one of the main symptoms of neurofibromatosis type 1 (NF1). As an important cognitive function, working memory (WM) has rarely been systematically analyzed in NF1 by isolating the particular domain of WM, and existing data involving WM in adult patients with NF1 are insufficient. This study aimed to investigate the characteristics of different types of WM in NF1 from the perspective of the adult population.Method: We comprehensively analyzed WM in both verbal and visuospatial WM domains by using the N-back task (including the verbal N-back task and the visuospatial N-back task) in 31 adults with NF1 and 34 healthy controls matched for age, gender, education levels, and general cognitive status. The accuracy and reaction times (RTs) in the N-back task were entered into mixed-design ANOVA.Results: Compared with healthy controls, adults with NF1 presented significantly lower mean accuracy and longer RTs in the visuospatial N-back task. However, no significant difference was found between the NF1 group and healthy controls in the verbal N-back task.Conclusions: The present study suggested that adults with NF1 might have deficits in visuospatial WM. We did not find evidence for verbal WM deficits in adult patients with NF1. Our findings supplement and refine the existing data on WM in the context of NF1.

Highlights

  • Neurofibromatosis type 1 (NF1) is an autosomal-dominant disorder with an average global prevalence of ∼1/3000 (Gutmann et al, 2017)

  • Based on the most influential model of working memory (WM) provided by Baddeley, WM can be subdivided into verbal WM and visuospatial WM (Baddeley, 2012), involving the temporary maintenance and manipulation of verbal and visuospatial information, respectively (Acheson and MacDonald, 2009; McAfoose and Baune, 2009)

  • Patients with NF1 were slower than the healthy controls, which was mainly caused by difficult levels of the task (i.e., 1-back and 2-back), as evidenced by a significant interaction effect on reaction times (RTs) [F(2,124) = 6.50, p = 0.002]

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Summary

Introduction

Neurofibromatosis type 1 (NF1) is an autosomal-dominant disorder with an average global prevalence of ∼1/3000 (Gutmann et al, 2017). Based on the most influential model of WM provided by Baddeley, WM can be subdivided into verbal WM and visuospatial WM (Baddeley, 2012), involving the temporary maintenance and manipulation of verbal and visuospatial information, respectively (Acheson and MacDonald, 2009; McAfoose and Baune, 2009). Their deficits will exert important effects on education- and work-related activities and affect the quality of life. This study aimed to investigate the characteristics of different types of WM in NF1 from the perspective of the adult population

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