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JNNP:亨廷顿病患者额叶纹状体回路对认知灵活性的影响:来自青年病人研究的证据

2020-11-22 MedSci原创 MedSci原创

亨廷顿氏病(HD)是一种遗传性、罕见的神经退行性疾病,以运动、认知和精神症状为特征。 HD是由亨廷顿蛋白基因(HTT)外显子1的三核苷酸胞嘧啶腺嘌呤鸟嘌呤(CAG)的重复扩张引起的,这导致亨廷顿蛋白的

亨廷顿氏病(HD)是一种遗传性、罕见的神经退行性疾病,以运动、认知和精神症状为特征。 HD是由亨廷顿蛋白基因(HTT)外显子1的三核苷酸胞嘧啶腺嘌呤鸟嘌呤(CAG)的重复扩张引起的,这导致亨廷顿蛋白的突变形式的表达。认知灵活性是适应性决策的关键,它与前额叶皮层(PFC)纹状体回路相联系,在显性和早发性亨廷顿病(pre-HD)中均受损。HD和HD前的纹状体神经退行性变尤其严重。这项研究的目的是检查一个远未发病的亨廷顿病(HD)队列的认知灵活性,以确定是否存在早期损伤,如果存在,研究额纹状体回路是否与此缺陷相关。

方法:在本研究中,作者检测了51名HD受试者(平均年龄=29.22(SD=5.71)岁)和53名年龄、性别和智商匹配的对照组,在剑桥神经心理学测试(CANTAB)内额外维度集移位(IED)任务中进行了测试。这个队列是唯一的,因为它是迄今为止最远离疾病发病年龄的综合研究(平均年=23.89(标准差=5.96)年)。IED任务是测量视觉辨别力,认知的灵活性,尤其是注意力的集中转移。使用静止状态功能性MRI检查与HD对照相比,研究特定前额纹状体回路之间的功能连通性在HD前是否异常。

结果:CANTAB-IED任务检测到轻度的早期认知灵活性损害。注意力转移与健康对照组腹外侧PFC和腹侧纹状体之间的功能连接以及HD前参与者的背外侧PFC和尾状体之间的功能连接显著相关。在远离疾病发作的HD前组中,CANTAB IED任务检测到认知灵活性的轻度损伤。大多数HD前样本的表现与对照组相当,但少数参与者表现较差。在健康个体中,腹外侧PFC和腹侧纹状体之间的功能连接与认知灵活性有关。在HD前组,交替的额-纹状体回路与注意定势转移有关,可能代表一种功能重组形式,虽然对大多数HD前参与者有效,但在少数受影响最大的HD前参与者中,这种方式不适应。因此,HD患者在早期认知功能障碍时的内在联系可能提供一个潜在的认知灵活性。

作者推测,这种认知灵活性的早期损害可能与HD前额叶纹状体回路内在的功能连接异常有关。

Langley CGregory SOsborne-Crowley K, et al Fronto-striatal circuits for cognitive flexibility in far from onset Huntington’s disease: evidence from the Young Adult Study .

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    2020-11-23 易水河

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