Schwabova J, Maly T, Laczo J, Zumrova A, Komarek V, Musova Z, Zahalka F
Department of Neurology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, V Uvalu 84, Prague 5, 15006, Czech Republic; Department of Paediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, V Uvalu 84, Prague 5, 15006, Czech Republic.
Research Sport Center, Charles University in Prague, Faculty of Physical Education and Sport, Jose Martiho 31, Prague 6, 16252, Czech Republic.
J Neurol Sci. 2014 Jun 15;341(1-2):64-7. doi: 10.1016/j.jns.2014.04.001. Epub 2014 Apr 12.
A scale for the Assessment and Rating of Ataxia (SARA) was developed for evaluation of autosomal dominant cerebellar ataxias (ADCA) and was also recommended for clinical trials of Friedreich's ataxia patients (FRDA). FRDA, unlike ADCA, is characterized as being a sensory type of ataxia for which the disease-specific Friedreich ataxia rating scale (FARS) was developed. The objective of this study was to determine whether SARA and FARS scores are associated with posturographic parameters in FRDA patients.
Adult patients with genetically confirmed FRDA (n=11) and ADCA (n=13) were evaluated by SARA, FARS and posturography.
FRDA patients' postural stability parameters, in stance with visual control, correlated with balance impairment in FARS (r=0.622; p<0.05) and SARA (r=0.735; p<0.05). Without visual control, only FARS correlated with balance impairment (r=0.732; p<0.05).
The SARA, in FRDA patients, correlates with stance with visual control but not without visual control which emphasizes sensory ataxia. This suggests that application of the SARA in Friedreich's ataxia patients according to posturography is possible but presumably limited and FARS, although being a more time consuming scale, may have advantages over SARA in FRDA patients.
共济失调评估与评分量表（SARA）是为评估常染色体显性遗传性小脑共济失调（ADCA）而制定的，也被推荐用于弗里德赖希共济失调患者（FRDA）的临床试验。与ADCA不同，FRDA的特征是感觉性共济失调，为此专门制定了疾病特异性的弗里德赖希共济失调评分量表（FARS）。本研究的目的是确定SARA和FARS评分是否与FRDA患者的姿势描记参数相关。
对经基因确诊的FRDA患者（n = 11）和ADCA患者（n = 13）进行SARA、FARS评估和姿势描记。
在视觉控制下站立时，FRDA患者的姿势稳定性参数与FARS（r = 0.622；p < 0.05）和SARA（r = 0.735；p < 0.05）的平衡损害相关。在无视觉控制时，只有FARS与平衡损害相关（r = 0.732；p < 0.05）。
在FRDA患者中，SARA与有视觉控制时的站立相关，但与无视觉控制时的站立无关，后者强调感觉性共济失调。这表明根据姿势描记法在弗里德赖希共济失调患者中应用SARA是可行的，但可能有局限性，而FARS虽然是一个耗时更长的量表，但在FRDA患者中可能比SARA更具优势。
