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

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.

Quantitative Assessment of Brain Stem and Cerebellar Atrophy in Spinocerebellar Ataxia Types 3 and 6: Impact on Clinical Status

“…The overall range of the total sum score is broader in ICARS than in SARA (100 versus 40 points), and rating results in all SARA items seem to be loaded on a single factor, whereas Ͼ1 factor is determined in the ICARS. 24,25 Hence, the SARA only rates ataxia-related symptoms. 25 This possibly leads to a higher contribution of cerebellar volume loss on the disease severity score, especially in diseases in which ataxia is dominant.…”

Comparing speech characteristics in spinocerebellar ataxias type 3 and type 6 with Friedreich ataxia

“…The three clinical groups were matched for two parameters, i.e., (1) disease severity (measured by the Scale for the Assessment and Rating of Ataxia (SARA) [14,15] administered by a neurologist not involved in the assessment of the dysarthria profile) and (2) disease duration (Table 1). One-way ANOVA revealed no significant between-group differences, neither for overall SARA-score (SARA total ; F (2, 27) = 0.238; p = 0.79) nor for disease duration (F (2, 27) = 0.371; p = 0.69).…”

Ophthalmic features of Friedreich ataxia

“…The ICARS scale evaluates two items related to visual function: postural and stance disturbances, and oculomotor dysfunction, whereas none of the items scored by the FARS scale are related to visual function. 19 Fortuna et al 1 found positive correlations between scores obtained in the ICARS test and disease duration, and mean RNFL thickness. However, they did not find a significant correlation with contrast sensitivity as we did.…”