Bluschke Annet, von der Hagen Maja, Papenhagen Katharina, Roessner Veit, Beste Christian
Cognitive Neurophysiology, Department of Child and Adolescent Psychiatry, Faculty of Medicine to the TU Dresden, Germany.
Abteilung Neuropädiatrie, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Germany.
Neuroimage Clin. 2017 Feb 20;14:499-505. doi: 10.1016/j.nicl.2017.02.014. eCollection 2017.
Neurofibromatosis Type 1 (NF1) is a monogenetic autosomal-dominant disorder with a broad spectrum of clinical symptoms and is commonly associated with cognitive deficits. Patients with NF1 frequently exhibit cognitive impairments like attention problems, working memory deficits and dysfunctional inhibitory control. The latter is also relevant for the resolution of cognitive conflicts. However, it is unclear how conflict monitoring processes are modulated in NF1. To examine this question in more detail, we used a system neurophysiological approach combining high-density ERP recordings with source localisation analyses in juvenile patients with NF1 and controls during a flanker task. Behaviourally, patients with NF1 perform significantly slower than controls. Specifically on trials with incompatible flanker-target pairings, however, the patients with NF1 made significantly fewer errors than healthy controls. Yet, importantly, this overall successful conflict resolution was reached via two different routes in the two groups. The healthy controls seem to arrive at a successful conflict monitoring performance through a developing conflict recognition via the N2 accompanied by a selectively enhanced N450 activation in the case of perceived flanker-target conflicts. The presumed dopamine deficiency in the patients with NF1 seems to result in a reduced ability to process conflicts via the N2. However, NF1 patients show an increased N450 irrespective of cognitive conflict. Activation differences in the orbitofrontal cortex (BA11) and anterior cingulate cortex (BA24) underlie these modulations. Taken together, juvenile patients with NF1 and juvenile healthy controls seem to accomplish conflict monitoring via two different cognitive neurophysiological pathways.
1型神经纤维瘤病（NF1）是一种单基因常染色体显性疾病，具有广泛的临床症状，通常与认知缺陷有关。NF1患者经常表现出认知障碍，如注意力问题、工作记忆缺陷和抑制控制功能障碍。后者也与认知冲突的解决有关。然而，目前尚不清楚NF1中冲突监测过程是如何被调节的。为了更详细地研究这个问题，我们采用了一种系统神经生理学方法，在侧翼任务期间，将高密度事件相关电位（ERP）记录与源定位分析相结合，对患有NF1的青少年患者和对照组进行研究。行为学上，NF1患者的表现明显比对照组慢。然而，具体在侧翼刺激与目标不匹配的试验中，NF1患者的错误明显少于健康对照组。然而，重要的是，两组通过两种不同的途径实现了总体上成功的冲突解决。健康对照组似乎通过N2伴随感知到的侧翼刺激与目标冲突时选择性增强的N450激活来发展冲突识别，从而达到成功的冲突监测表现。NF1患者中推测的多巴胺缺乏似乎导致通过N2处理冲突的能力下降。然而，无论认知冲突如何，NF1患者的N450都会增加。眶额皮质（BA11）和前扣带回皮质（BA24）的激活差异是这些调节的基础。综上所述，患有NF1的青少年患者和青少年健康对照组似乎通过两种不同的认知神经生理途径完成冲突监测。
