Cognitive function and its relationship with brain structure in myotonic dystrophy type 1.,Journal of Neuroscience Research

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Cognitive function and its relationship with brain structure in myotonic dystrophy type 1.
Journal of Neuroscience Research ( IF 2.9 ) Pub Date : 2020-02-13 , DOI: 10.1002/jnr.24595
Kathleen E Langbehn ₁ , Ellen van der Plas ₁ , David J Moser ₁ , Jeffrey D Long ₁ , Laurie Gutmann ₂ , Peggy C Nopoulos _{1,

2}

Affiliation

Studies have shown relationships between white matter abnormalities and cognitive dysfunction in myotonic dystrophy type 1 (DM1), but comprehensive analysis of potential structure–function relationships are lacking. Fifty adult‐onset DM1 individuals (33 female) and 68 unaffected adults (45 female) completed the Wechsler Adult Intelligence Scale‐IV (WAIS‐IV) to determine the levels and patterns of intellectual functioning. Neuroimages were acquired with a 3T scanner and were processed with BrainsTools. Regional brain volumes (regions of interest, ROIs) were adjusted for inter‐scanner variation and intracranial volume. Linear regression models were conducted to assess if group by ROI interaction terms significantly predicted WAIS‐IV composite scores. Models were adjusted for age and sex. The DM1 group had lower Perceptual Reasoning Index (PRI), Working Memory Index (WMI), and Processing Speed Index (PSI) scores than the unaffected group (PRI t₍₁₁₃₎ = −3.28, p = 0.0014; WMI t₍₁₁₄₎ = −3.49, p = 0.0007; PSI t₍₁₁₄₎ = −2.98, p = 0.0035). The group by hippocampus interaction term was significant for both PRI and PSI (PRI (t₍₁₁₁₎ = −2.82, p = 0.0057; PSI (t₍₁₁₂₎ = −2.87, p = 0.0049)). There was an inverse association between hippocampal volume and both PRI and PSI in the DM1 group (the higher the volume, the lower the intelligence quotient scores), but no such association was observed in the unaffected group. Enlarged hippocampal volume may underlie some aspects of cognitive dysfunction in adult‐onset DM1, suggesting that increased volume of the hippocampus may be pathological.

中文翻译：

1型肌强直性营养不良的认知功能及其与脑结构的关系。

研究表明 1 型肌强直性营养不良 (DM1) 的白质异常与认知功能障碍之间存在关系，但缺乏对潜在结构 - 功能关系的综合分析。50 名成年 DM1 个体（33 名女性）和 68 名未受影响的成年人（45 名女性）完成了韦克斯勒成人智力量表-IV (WAIS-IV) 以确定智力功能的水平和模式。使用 3T 扫描仪获取神经图像并使用 BrainsTools 进行处理。区域脑容量（感兴趣区域，ROI）根据扫描仪间的变化和颅内容量进行了调整。进行线性回归模型以评估按 ROI 交互项分组是否显着预测 WAIS-IV 综合得分。模型根据年龄和性别进行了调整。DM1 组的感知推理指数 (PRI) 较低，t ₍₁₁₃₎ = -3.28, p = 0.0014; WMI t ₍₁₁₄₎ = -3.49，p = 0.0007；PSI t ₍₁₁₄₎ = -2.98，p = 0.0035）。海马相互作用项组对 PRI 和 PSI 均显着（PRI ( t ₍₁₁₁₎ = -2.82 , p = 0.0057; PSI ( t ₍₁₁₂₎ = -2.87, p = 0.0049))。DM1 组海马体积与 PRI 和 PSI 之间存在负相关（体积越大，智商得分越低），但在未受影响的组中未观察到这种关联。海马体积扩大可能是成人发病 DM1 认知功能障碍的某些方面的基础，这表明海马体积增加可能是病理性的。

更新日期：2020-02-13

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