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患有眼阵挛-肌阵挛综合征的儿童和青少年大脑功能连接改变。

Altered functional brain connectivity in children and young people with opsoclonus-myoclonus syndrome.

作者信息

Chekroud Adam M, Anand Geetha, Yong Jean, Pike Michael, Bridge Holly

机构信息

FMRIB Centre, Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.

Oxford Children's Hospital, John Radcliffe Hospital, Oxford, UK.

出版信息

Dev Med Child Neurol. 2017 Jan;59(1):98-104. doi: 10.1111/dmcn.13262. Epub 2016 Sep 23.

DOI:10.1111/dmcn.13262
PMID:27658927
Abstract

AIM

Opsoclonus-myoclonus syndrome (OMS) is a rare, poorly understood condition that can result in long-term cognitive, behavioural, and motor sequelae. Several studies have investigated structural brain changes associated with this condition, but little is known about changes in function. This study aimed to investigate changes in brain functional connectivity in patients with OMS.

METHOD

Seven patients with OMS and 10 age-matched comparison participants underwent 3T magnetic resonance imaging (MRI) to acquire resting-state functional MRI data (whole-brain echo-planar images; 2mm isotropic voxels; multiband factor ×2) for a cross-sectional study. A seed-based analysis identified brain regions in which signal changes over time correlated with the cerebellum. Model-free analysis was used to determine brain networks showing altered connectivity.

RESULTS

In patients with OMS, the motor cortex showed significantly reduced connectivity, and the occipito-parietal region significantly increased connectivity with the cerebellum relative to the comparison group. A model-free analysis also showed extensive connectivity within a visual network, including the cerebellum and basal ganglia, not present in the comparison group. No other networks showed any differences between groups.

INTERPRETATION

Patients with OMS showed reduced connectivity between the cerebellum and motor cortex, but increased connectivity with occipito-parietal regions. This pattern of change supports widespread brain involvement in OMS.

摘要

目的

眼阵挛-肌阵挛综合征(OMS)是一种罕见且了解甚少的病症,可导致长期的认知、行为和运动后遗症。多项研究调查了与该病症相关的脑结构变化,但对功能变化知之甚少。本研究旨在调查OMS患者脑功能连接的变化。

方法

7名OMS患者和10名年龄匹配的对照参与者接受了3T磁共振成像(MRI),以获取静息态功能MRI数据(全脑回波平面图像;各向同性体素2mm;多频段因子×2)用于横断面研究。基于种子点的分析确定了信号随时间变化与小脑相关的脑区。采用无模型分析来确定连接性改变的脑网络。

结果

与对照组相比,OMS患者的运动皮层连接性显著降低,枕顶叶区域与小脑的连接性显著增加。无模型分析还显示,在一个视觉网络内存在广泛的连接,包括小脑和基底神经节,而对照组不存在这种情况。其他网络在两组之间未显示任何差异。

解读

OMS患者小脑与运动皮层之间的连接性降低,但与枕顶叶区域的连接性增加。这种变化模式支持OMS存在广泛的脑受累情况。

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