Objective

Spinocerebellar ataxia (SCA) is a neurodegenerative disorder in which, autonomic dysfunction is a common manifestation. Brain area atrophy also involves the areas comprising central autonomic network (CAN) in SCA. Structural atrophy of CAN and autonomic dysfunction should go hand in hand. But this important relationship has not been studied to date. Therefore, using SCA as a disease model, the present study has been designed to explore the plausible correlations between the brain areas of CAN and clinical autonomic function modalities in SCA patients.

Materials and methods

3D T1-weighted scans were acquired on 3T MRI, analyzed by FreeSurfer software in genetically confirmed forty-nine SCA patients (SCA1 = 18, SCA2 = 25 and SCA3 = 6). Heart rate variability (HRV), blood pressure variability (BPV), baroreflex sensitivity (BRS), and autonomic reactivity tests were used for evaluation of autonomic nervous system. Additionally, autonomic dysfunction scoring was done using composite autonomic severity score (CASS).

Results

On correlation analysis, the study showed the association of atrophic cortical and subcortical brain areas (predominantly prefrontal cortex, bilateral middle temporal, left cuneus, left lingual and left caudate) with altered clinical autonomic function parameters in SCA patients. These areas were primarily comprised of sympathetic and parasympathetic brain areas of CAN. One of the key brain areas of CAN – left cuneus was found to be associated with both HRV (r = 0.295, p = 0.040) and BRS (r = 0.326, p = 0.022).

Conclusion

A characteristic pattern of association between particular brain areas of CAN and clinical autonomic function parameters was observed in SCA patients.

中文翻译：

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脊髓小脑性共济失调患者中枢自主神经网络结构性萎缩与自主神经系统功能特性的相关性

客观的

脊髓小脑性共济失调（SCA）是一种神经退行性疾病，其中自主神经功能障碍是常见的表现。脑区萎缩还涉及 SCA 中包含中枢自主神经网络 (CAN) 的区域。CAN 的结构性萎缩和自主神经功能障碍应该齐头并进。但迄今为止，尚未研究这种重要的关系。因此，使用 SCA 作为疾病模型，本研究旨在探索 CAN 大脑区域与 SCA 患者临床自主神经功能模式之间的似是而非的相关性。

材料和方法

3D T1 加权扫描是在 3T MRI 上获得的，通过 FreeSurfer 软件在基因确认的 49 名 SCA 患者（SCA1 = 18、SCA2 = 25 和 SCA3 = 6）中进行分析。心率变异性 (HRV)、血压变异性 (BPV)、压力反射敏感性 (BRS) 和自主神经反应测试用于评估自主神经系统。此外，使用综合自主神经严重程度评分（CASS）进行自主神经功能障碍评分。

结果

在相关性分析中，该研究表明萎缩性皮质和皮质下脑区（主要是前额叶皮质、双侧中颞叶、左楔叶、左舌和左尾状核）与 SCA 患者临床自主神经功能参数改变之间存在关联。这些区域主要由 CAN 的交感神经和副交感神经脑区组成。发现 CAN 的关键大脑区域之一 - 左楔骨与 HRV (r = 0.295, p = 0.040) 和 BRS (r = 0.326, p = 0.022) 相关。

结论

在 SCA 患者中观察到 CAN 特定脑区与临床自主神经功能参数之间关联的特征模式。