三种临床评定量表在弗里德赖希共济失调（FRDA）中的比较。

Comparison of three clinical rating scales in Friedreich ataxia (FRDA).

作者信息

Bürk Katrin, Mälzig Ulrike, Wolf Stefanie, Heck Suzette, Dimitriadis Konstantinos, Schmitz-Hübsch Tanja, Hering Sascha, Lindig Tobias M, Haug Verena, Timmann Dagmar, Degen Ingrid, Kruse Bernd, Dörr Jan-Markus, Ratzka Susanne, Ivo Anja, Schöls Ludger, Boesch Sylvia, Klockgether Thomas, Klopstock Thomas, Schulz Jörg B

机构信息

Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen, Göttingen, Germany.

出版信息

Mov Disord. 2009 Sep 15;24(12):1779-84. doi: 10.1002/mds.22660.

DOI:10.1002/mds.22660

PMID:19562766

Abstract

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 forclinical trials of FRDA.

摘要

为检验共济失调评估与分级量表（SARA）在弗里德赖希共济失调（FRDA）中的有效性和可靠性。SARA仅包含八个项目，可快速完成。使用三种不同的临床量表，即FRDA分级量表（FARS）、国际协作共济失调分级量表（ICARS）和SARA，对96例经分子遗传学诊断为FRDA的患者进行评分。尽管量表规模和子量表结构存在显著差异，但SARA总分与ICARS总分（r = 0.953，P < 0.0001）和FARS总分（r = 0.938，P < 0.0001）显著相关。SARA总分也与日常生活活动能力（ADL，r = 0.929，P < 0.0001）相关。尽管SARA最初是为主要用于小脑型的显性遗传性共济失调而开发的，但它也可以成功地用于评估传入性共济失调，这是FRDA中的主要形式。由于SARA具有较高的评分者间信度和实用性，因此适用于FRDA的临床试验。