Comparison of Three Clinical Rating Scales In Friedreich Ataxia (FRDA)

Comparison of Three Clinical Rating Scales In Friedreich Ataxia (FRDA)
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DOI:
10.1002/mds.22660
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发表时间:
2009-09-15
期刊:
影响因子:
8.6
通讯作者:
Schulz, Joerg B.
Schulz, Joerg B.
中科院分区:
医学1区
文献类型:
--
作者:
Buerk, Katrin;Maelzig, Ulrike;Schulz, Joerg B.

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目的:检验共济失调评定量表(SARA)在弗里德赖希共济失调(FRDA)中的信度和效度。SARA仅限于8个项目,可以快速执行。采用三种不同的临床量表(FRDA评定量表(法尔斯)、国际合作共济失调评定量表(ICARS)和SARA)对96例分子遗传学诊断为FRDA的患者进行评定。尽管在量表大小和子量表结构上存在相当大的差异。SARA总分与ICARS总分(r = 0.953,P < 0.0001)和法尔斯总分(r = 0.938,P < 0.0001)显著相关。SARA总分与日常生活活动能力(ADL,r = 0.929,P < 0.0001)相关。虽然最初开发用于显性遗传性共济失调(主要是小脑型共济失调),但SARA也可成功用于评估传入性共济失调,这是FRDA的主要形式。由于SARA具有较高的评价者间可靠性和实用性,因此SARA适用于FRDA的临床试验。(C)2009运动障碍协会
To test the validity and reliability of the scale for the assessment and rating of ataxia (SARA) in Friedreich ataxia (FRDA). SARA is limited to eight items and can be performed rapidly. Ninety-six patients with a molecular genetic diagnosis of FRDA were rated using three different clinical scales, the FRDA Rating Scale (FARS), the International Cooperative Ataxia Rating Scale (ICARS), and SARA. Despite considerable discrepancies in scale size and subscale structure. SARA total scores were significantly correlated with ICARS (r = 0.953, P < 0.0001) and FARS (r = 0.938, P < 0.0001) total scores. SARA total scores also correlated with the activities of daily living (ADL, r = 0.929, P < 0.0001). Although originally developed for the use in dominantly inherited ataxias, which are primarily ataxias of the cerebellar type, SARA can also be used Successfully to assess afferent ataxia, which is the predominant form in FRDA. Because SARA is characterized by high interrater reliability and practicability, SARA is applicable and well suited for clinical trials of FRDA. (C) 2009 Movement Disorder Society