Quantitative analysis of upper-limb ataxia in patients with spinocerebellar degeneration
Quantitative analysis of upper-limb ataxia in patients with spinocerebellar degeneration
复制标题
脊髓小脑变性患者上肢共济失调的定量分析
DOI：
10.1007/s00415-014-7353-4
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发表时间：
2014
影响因子：
6
通讯作者：
Fumiaki Tanaka
中科院分区：
文献类型：
--
作者：
Naohisa Ueda;Yasuhito Hakii;Shigeru Koyano;Yuichi Higashiyama;Hideto Joki;Yasuhisa Baba;Yume Suzuki;Yoshiyuki Kuroiwa;Fumiaki Tanaka
脊髓小脑变性（SCD）是一种进行性神经退行性疾病，其中小脑共济失调导致运动障碍。没有广泛适用的方法来客观评价SCD中的共济失调。一个客观的系统来评估共济失调是必要的，用于新开发的药物和康复的临床试验。本研究的目的是开发一种简单的方法来量化上肢共济失调的程度。49例SCD患者参加了本研究。指导患者追踪阿基米德螺旋模板，并使用Image J软件测量模板螺旋和绘制螺旋之间的差距（间隙面积; GA）。使用共济失调评估和评级量表（SARA）对共济失调进行评级，并使用临床评价6个月内获得的磁共振图像的轴向横截面对37例患者的小脑体积进行评价。进行回归分析以评估GA与患者年龄、病程、SARA评分和小脑体积之间的关系。GA与SARA总分显著相关（r = 0.660，p <0.001），姿势和步态（r = 0.551，p <0.001），言语（r = 0.527，p <0.001），手部运动（r = 0.553，p <0.001），跟胫滑动（r = 0.367，p = 0.036）SARA亚评分和小脑体积（r = 0.577，p <0.001），但与患者年龄（r = 0.176，p = 0.227）和病程（r = 0.236，p = 0.103）无关。GA是一种简单、有用的方法，可以客观地量化小脑共济失调，特别是上肢共济失调的程度，并可广泛用于各种环境，包括临床试验。
Spinocerebellar degeneration (SCD) is a progressive neurodegenerative disorder in which cerebellar ataxia causes motor disability. There are no widely applicable methods for objective evaluation of ataxia in SCD. An objective system to evaluate ataxia is necessary for use in clinical trials of newly developed medication and rehabilitation. The aim of this study was to develop a simple method to quantify the degree of upper-limb ataxia. Forty-nine patients with SCD participated in this study. Patients were instructed to trace an Archimedean spiral template, and the gap between the template spiral and the drawn spiral (gap area; GA) was measured using Image J software. Ataxia was rated using the Scale for the Assessment and Rating of Ataxia (SARA) and cerebellar volume was evaluated in 37 patients using an axial cross-section of magnetic resonance images that were obtained within 6 months of clinical evaluation. Regression analysis was performed to assess the relation between GA and patient age, disease duration, SARA score, and cerebellar volume. GA was significantly related to total SARA score (r= 0.660,p< 0.001), the posture and gait (r= 0.551,p< 0.001), speech (r= 0.527,p< 0.001), hand movements (r= 0.553,p< 0.001), and heel-shin slide (r= 0.367,p= 0.036) SARA subscores, and cerebellar volume (r= 0.577,p< 0.001) but was not related to patient age (r= 0.176,p= 0.227) or disease duration (r= 0.236,p= 0.103). GA is a simple, useful method to objectively quantify the degree of cerebellar ataxia, especially upper-limb ataxia, and can be widely adopted in various settings, including clinical trials.