Department of Rehabilitation Medicine, The First Affiliated Hospital, Fujian Medical University, Fuzhou, China.
Fujian Medical University, Fuzhou, China.
Ann Clin Transl Neurol. 2020 Aug;7(8):1360-1370. doi: 10.1002/acn3.51124. Epub 2020 Jul 7.
Spinocerebellar ataxia type 3 (SCA3) is one of the most common hereditary neurodegenerative diseases, with balance instability as main symptom. Balance quantification is crucial for evaluating the efficacy of therapeutic interventions. However, balance evaluation in SCA3 is often subject to bias. Here, we aimed to quantitatively evaluate postural instability and investigate the relationship between postural instability and clinical characteristics in SCA3 patients.
Sixty-two SCA3 patients and 62 normal controls were recruited, and their postural balance was measured using a posturographic platform. Principal component analysis was performed as data reduction to identify postural instability factors. Multivariable linear regression was used to investigate potential risk factors for postural instability and to explore whether postural instability predicts the severity and progression of ataxia in SCA3 patients.
We found SCA3 patients experience postural instability characterized by significant impairment in static and dynamic stability. The condition without visual feedback was the most sensitive measure in differentiating SCA3 from controls. Regression analyses revealed that ataxia severity predicted both static (P = 0.014) and dynamic stability (P = 0.001). Likewise, along with expanded CAG repeats (P < 0.001), both static (P < 0.001) and dynamic stability (P < 0.001) predicted ataxia severity, but not ataxia progression.
Our findings demonstrate the validity of using the Pro-kin system for assessing postural instability in SCA3 patients. This type of quantitative assessment of balance dysfunction can contribute to clinical trials and balance rehabilitation in SCA3 patients.
脊髓小脑性共济失调 3 型（SCA3）是最常见的遗传性神经退行性疾病之一，以平衡不稳为主要症状。平衡量化对于评估治疗干预的疗效至关重要。然而，SCA3 的平衡评估常常存在偏差。在此，我们旨在定量评估姿势不稳，并研究 SCA3 患者中姿势不稳与临床特征之间的关系。
招募了 62 名 SCA3 患者和 62 名正常对照者，并使用平衡测试平台测量其姿势平衡。采用主成分分析作为数据降维方法，以识别姿势不稳因素。采用多元线性回归分析潜在的姿势不稳危险因素，并探讨姿势不稳是否预测 SCA3 患者的共济失调严重程度和进展。
我们发现 SCA3 患者存在姿势不稳，表现为静态和动态稳定性显著受损。无视觉反馈的情况是区分 SCA3 与对照组的最敏感测量方法。回归分析显示，共济失调严重程度预测了静态（P=0.014）和动态稳定性（P=0.001）。同样，扩展的 CAG 重复（P<0.001）、静态（P<0.001）和动态稳定性（P<0.001）均预测了共济失调严重程度，但不能预测共济失调进展。
我们的研究结果证明了使用 Pro-kin 系统评估 SCA3 患者姿势不稳的有效性。这种平衡功能的定量评估方法可以为 SCA3 患者的临床试验和平衡康复提供帮助。
