Development and validation of a new ataxia rating scale: Scale for the Assessment and Rating of Ataxia (SARA)

Schmitz‐Hübsch, Tanja; Montcel, Sophie Tezenas du; Baliko, L.; Berciano, José; Boesch, Sylvia; Dürr, Alexandra; Giunti, Paola; Kang, Joseph W.; Mariotti, Caterina; Pandolfo, Massimo; Rakowicz, Maria; Schöls, Lüdger; Szymanski, Sandra; Warrenburg, Bart van de; Klockgether, Thomas

doi:10.1055/s-2005-919541

Cited by 6 publications

(16 citation statements)

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“…However, we evaluate cerebellar cortex disordered disease, cerebellar nucleus disordered disease, and feed-back pathway disordered disease using the same evaluation scale. SARA is a scale constructed to assess various types of SCD [1]. Study of the natural course of SCD using SARA indicates that the type of SCD or the different repeat number results in a different deterioration rate [13].…”

Section: Discussionmentioning

confidence: 99%

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Quantitative evaluation of gait ataxia by accelerometers

Shirai

Yabe

Matsushima

et al. 2015

Journal of the Neurological Sciences

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Correspondence to Hidenao Sasaki, h-isasak@med.hokudai.ac.jp Although there are various types of biomarkers such as molecular markers and imaging markers, an appropriate maker for neurodegenerative disease has not been identified. Thus, we use clinical evaluation scales. However, it is important to identify an appropriate biomarker in order to evaluate the efficiency of a given clinical trial.Here, we performed gait analyses in patients with pure cerebellar type spinocerebellar degeneration (SCD) and assessed whether the obtained data could be used as a neurophysiological biomarker for cerebellar ataxia.Here we performed gait analysis in patients with pure cerebellar type spinocerebellar degeneration (SCD) and assessed whether the obtained data could be used as a neurophysiological biomarker for cerebellar ataxia. We evaluated the clinical severity of each patient in the SCD group using the Scale for the Assessment and Rating of Ataxia (SARA) [1, 2] and the Berg Balance Scale (BBS) [3,4]. The mean SARA of the SCD group was 13.26±4.43, while the mean Unified Parkinson's Disease Rating Scale (UPDRS) part III was 18. 48±8.4760. 6 This study was approved by the ethics panels of Hokkaido University Hospital and Kushiro Rosai Hospital. Methods (Fig. 1)Acceleration signals were measured during 6 min walking and 1 min standing tasks by two sets of triaxial accelerometers (Mimamori-gait system, LSI Medience; size, 7.5 cm × 5 cm × 2 cm; weight, 95 g) that were secured with a fixation vest to the middle of the subject's lower and upper back.When in the standing position, the subjects were evaluated with their eyes open for 1 min and then their eyes closed for another 1 min. An assistant remained beside the subjects in order to prevent falls, however, no falls occurred. The subjects were evaluated while they shuttle-walked a 30 m straight line for 6 min (6-min walk test (6MWT) [5]). Similar tasks were performed in the JASMITT study [6] and in other studies. When standing in the anatomical position, the orientation of the three acceleration axes, X, Y, and Z, were medial/lateral (ML), vertical (VT), and anterior/posterior (AP), respectively. Data were collected at a sampling frequency of 100 Hz and stored on a secure digital memory card inserted into the device for later analysis. To quantify body motion during standing, we examined parameters as follows:1. The three acceleration components ax(t), ay(t), and az(t) were smoothed by a moving-window average with a window size of 5.6 s to generate three 8 baseline signals: Bx(t), By(t), and Bz(t). Statistical analysisWe used Student's t-test for inter-group comparison and Pearson's correlation coefficients for disease severity and each parameter analysis.The JMPⓇ Pro 11.2.0 software program (SAS Institute Inc., Cary, NC, USA) was used for statistical analyses. We considered the results significant if p<0.05. The test of significance was adjusted for multiple testing using a Bonferroni step-down (Holm) correction. ResultsWe compared each parameter of SCD patients to that of...

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Section: Discussionmentioning

confidence: 99%

“…We evaluated the clinical severity of each patient in the SCD group using the Scale for the Assessment and Rating of Ataxia (SARA) [1,2] and the Berg Balance Scale (BBS) [3,4]. The mean SARA of the SCD group was 13.26±4.43, while the mean Unified Parkinson's Disease Rating Scale (UPDRS) part III was 18.48±8.4760.…”

Section: Subjectsmentioning

confidence: 99%

Quantitative evaluation of gait ataxia by accelerometers

Shirai

Yabe

Matsushima

et al. 2015

Journal of the Neurological Sciences

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“…The scale was created in 1997 by the World Federation of Neurology Committee to provide a standardized clinical classification system to quantify deficits caused by cerebellar ataxia 11 . The ICARS has already been validated (English version) for evaluation of patients with multiple system atrophy and Parkinson's disease 23 , SCAs and Friedreich's ataxia 18,24,25 and focal cerebellar lesions 26 .…”

Section: Discussionmentioning

confidence: 99%

“…The use of the ICARS in clinical practice in patients with cerebellar ataxias has been criticized among professionals for being too long and having a great number of questions 27 . Indeed, Schmitz-Hubsch and coworkers 18 evaluated the metric properties of the ICARS. The scale was described as very long for application by the health professionals, with an average estimated time of 21 minutes.…”

Section: Discussionmentioning

confidence: 99%

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Cross-cultural adaptation and validation of the International Cooperative Ataxia Rating Scale (ICARS) to Brazilian Portuguese

Maggi

Braga‐Neto

Chien

et al. 2018

Arq. Neuro-Psiquiatr.

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Introduction: The clinical assessment of patients with ataxias requires reliable scales. We aimed to translate, adapt and validate the International Cooperative Ataxia Rating Scale (ICARS) into Brazilian Portuguese. Methods: The steps of this study were forward translation, translation synthesis, backward translation, expert committee meeting, preliminary pilot testing and final assessment. Thirty patients were enrolled in the preliminary pilot testing and 61 patients were evaluated for construct validity, internal consistency, intra- and inter-rater reliability and external consistency. Results: This study showed good validity of the construct and high internal consistency for the full scale, except for the oculomotor domain (Cronbach's alpha = 0.316, intraclass correlation coefficients intra- = 82.4% and inter- = 79.2%). A high correlation with the Scale for the Assessment and Rating of Ataxia was observed. We found good intra-rater agreement and relative inter-rater disagreement, except in the posture and gait domain. Conclusion: The present ICARS version is adapted for the Brazilian culture and can be used to assess our ataxic patients.

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Dance Improves Motor, Cognitive, and Social Skills in Children With Developmental Cerebellar Anomalies

et al. 2021

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In this multiple single-cases study, we used dance to train Sensorimotor Synchronization (SMS), motor and cognitive functions in children with Developmental Cerebellar Anomalies (DCA). DCA are rare dysfunctions of the cerebellum that affect motor and cognitive skills. The cerebellum plays an important role in temporal cognition including SMS which is, critical for motor and cognitive development. Dancing engages the SMS neuronal circuitry, composed of the cerebellum, the basal ganglia and the motor cortices. Thus, we hypothesized that dance has a beneficial effect on SMS skills and associated motor and cognitive functions in children with DCA. Seven children (aged 7-11) with DCA participated in a 2-month dance training protocol (3h/week). A test-retest design protocol with multiple baselines was used to assess children's SMS skills as well as motor, cognitive and social abilities. SMS skills were impaired in DCA before the training. The training led to improvements in SMS (reduced variability in paced tapping), balance and executive functioning (cognitive flexibility), as well as in social skills (social cognition). The beneficial effects of the dance training were visible in all participants. Notably, gains were maintained two months after the intervention. These effects are likely to be sustained by enhanced activity in SMS brain networks due to the dance training protocol.

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Development and validation of a new ataxia rating scale: Scale for the Assessment and Rating of Ataxia (SARA)

Cited by 6 publications

References 0 publications

Quantitative evaluation of gait ataxia by accelerometers

Quantitative evaluation of gait ataxia by accelerometers

Cross-cultural adaptation and validation of the International Cooperative Ataxia Rating Scale (ICARS) to Brazilian Portuguese

Dance Improves Motor, Cognitive, and Social Skills in Children With Developmental Cerebellar Anomalies

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