Brief Ataxia Rating Scale: A Reliable Tool to Rate Ataxia in a Short Timeframe

Camargos, Sarah Teixeira; Cardoso, Francisco; Maciel, Ricardo; Huebra, Lucio; Silva, Thiago Roberto; Campos, Vilson Geraldo; Alencar, Rodrigo

doi:10.1002/mdc3.12364

Cited by 11 publications

(5 citation statements)

References 9 publications

(10 reference statements)

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“…The BARS was developed to overcome some of the main limitations of other ataxia scales, such as length and redundancies 9 . The scale is completed by a trained health care professional, and it may take 3 to 5 minutes to complete 10 . The scale is not copyrighted and can be found in the original publication 9 …”

Section: Resultsmentioning

confidence: 99%

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Assessment of Ataxia Rating Scales and Cerebellar Functional Tests: Critique and Recommendations

Pérez-Lloret

Warrenburg

Rossi³

et al. 2020

Movement Disorders

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A BS TRACT: Background: We assessed the clinimetric properties of ataxia rating scales and functional tests, and made recommendations regarding their use. Methods: A systematic literature search was conducted to identify the instruments used to rate ataxia symptoms. The identified rating scales and functional ability tests were reviewed and ranked by the panel as "recommended," "suggested," or "listed" for the assessment of patients with discrete cerebellar disorders, using previously established criteria. Results: We reviewed 14 instruments (9 rating scales and 5 functional tests).

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Section: Resultsmentioning

confidence: 99%

“…9 The scale is completed by a trained health care professional, and it may take 3 to 5 minutes to complete. 10 The scale is not copyrighted and can be found in the original publication. 9 Clinimetric Properties There is no information on missingness and floor or ceiling effects.…”

Section: Rating Scalesmentioning

confidence: 99%

Assessment of Ataxia Rating Scales and Cerebellar Functional Tests: Critique and Recommendations

Pérez-Lloret

Warrenburg

Rossi³

et al. 2020

Movement Disorders

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“…An ataxia-specialist neurologist (A.S.G.) performed the Brief Ataxia Rating Scale (BARS) 21 using the half-point version, 22 the Scale for the Assessment and Rating of Ataxia (SARA) 23 and Part III of the Movement Disorder Society-Unified Parkinson Disease Rating Scale (MDS-UPDRS). 24 The sitting component of SARA and six rigidity components from UPDRS were excluded owing to the rater’s inability to perform/assess these tasks remotely.…”

Section: Methodsmentioning

confidence: 99%

Real-life ankle submovements and computer mouse use reflect patient-reported function in adult ataxias

Eklund

Ouillon

Pandey

et al. 2023

Brain Communications

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Novel disease modifying therapies are being evaluated in spinocerebellar ataxias and multiple system atrophy. Clinician-performed disease rating scales are relatively insensitive for measuring disease change over time, resulting in large and long clinical trials. We tested the hypothesis that sensors worn continuously at home during natural behavior and a web-based computer mouse task performed at home could produce interpretable, meaningful, and reliable motor measures for potential use in clinical trials. Thirty-four individuals with degenerative ataxias (spinocerebellar ataxia types 1, 2, 3, and 6 and multiple system atrophy of the cerebellar type) and eight age-matched controls completed the cross-sectional study. Participants wore an ankle and wrist sensor continuously at-home for one week and completed the Hevelius computer mouse task eight times over four weeks. We examined properties of motor primitives called “submovements” derived from the continuous wearable sensors and properties of computer mouse clicks and trajectories in relationship to patient-reported measures of function (PROM-Ataxia) and ataxia rating scales (Scale for the Assessment and Rating of Ataxia and the Brief Ataxia Rating Scale). The test-retest reliability of digital measures and differences between ataxia and control participants were evaluated. Individuals with ataxia had smaller, slower, and less powerful ankle submovements during natural behavior at home. A composite measure based on ankle submovements strongly correlated with ataxia rating scale scores (Pearson’s r = 0.82-0.88), strongly correlated with self-reported function (r = 0.81), had high test-retest reliability (intraclass correlation coefficient = 0.95), and distinguished ataxia and control participants, including preataxic individuals (N=4) from controls. A composite measure based on computer mouse movements and clicks strongly correlated with ataxia rating scale total (r = 0.86-0.88) and arm scores (r = 0.65-0.75), correlated well with self-reported function (r = 0.72-0.73), and had high test-retest reliability (intraclass correlation coefficient = 0.99). These data indicate that interpretable, meaningful, and highly reliable motor measures can be obtained from continuous measurement of natural movement, particularly at the ankle location, and from computer mouse movements during a simple point-and-click task performed at home. This study supports the use of these two inexpensive and easy-to-use technologies in longitudinal natural history studies in spinocerebellar ataxias and multiple system atrophy of the cerebellar type and shows promise as potential motor outcome measures in interventional trials.

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“…The BARS is tightly correlated with the Scale for Assessment and Rating of Ataxia 20 and the International Cooperate Ataxia Rating Scale, 21 and has been replicated and validated. 22 , 23 A revised version with half-point measures describing deficits with greater granularity has been validated in clinical assessments 24 , 25 and in quantitative observer-independent sensor studies. 26-31 In late MSA-C, parkinsonism can become severe and mask ataxia.…”

Section: Methodsmentioning

confidence: 99%

Rates of change of pons and middle cerebellar peduncle diameters are diagnostic of multiple system atrophy of the cerebellar type

Stephen,

Vangel,

Gupta

et al. 2023

Brain Communications

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Definitive diagnosis of multiple system atrophy of the cerebellar type (MSA-C) is challenging. We hypothesized that rates of change of pons and middle cerebellar peduncle (MCP) diameters on MRI would be unique to MSA-C and serve as diagnostic biomarkers. We defined the normative data for anterior-posterior (AP) pons and transverse MCP diameters on brain MRI in healthy controls, performed diameter-volume correlations, and measured intra-and inter-rater reliability. We studied an Exploratory cohort (2002-2014) of 88 MSA-C and 78 other cerebellar ataxia patients, and a Validation cohort (2015-2021) of 49 MSA-C, 13 MSA-P, 99 other cerebellar ataxia patients, and 314 non-ataxia patients. We measured AP Pons and MCP diameters on baseline and subsequent MRIs, and correlated results with Brief Ataxia Rating Scale scores. We assessed midbrain:pons and MCP:pons ratios over time. The normative AP Pons diameter was 23.6±1.6 mm, and MCP diameter 16.4±1.4 mm. Pons diameter correlated with volume, r=0.94, p<0.0001. The AP Pons and MCP measures were smaller at first scan in MSA-C compared to all other ataxias. AP Pons diameter: Exploratory, 19.3±2.6 mm vs. 20.7±2.6 mm, Validation, 19.9±2.1 mm vs 21.1±2.1 mm. MCP transverse diameter, Exploratory, 12.0±2.6 mm vs. 14.3±2.1 mm, Validation, 13.6±2.1 mm vs 15.1±1.8 mm, all p<0.001. The AP Pons and MCP rates of change were faster in MSA-C than in all other ataxias. AP Pons diameter rates of change: Exploratory, -0.87±0.04 mm/year vs. -0.09±0.02 mm/year, Validation, -0.89±0.48 mm/year vs. -0.10±0.21 mm/year. MCP transverse diameter rates of change: Exploratory, -0.84±0.05 mm/year vs. -0.08±0.02 mm/year, Validation, -0.94±0.64 mm/year vs -0.11±0.27 mm/year, all values p<0.0001. AP Pons and MCP diameters were indistinguishable between Possible, Probable, and Definite MSA-C. The rate of AP Pons atrophy was linear, correlating with ataxia severity. Using a lower threshold AP Pons diameter decrease of -0.4 mm/year to balance sensitivity and specificity, area under the curve analysis (AUC) discriminating MSA-C from other ataxias was 0.94, yielding sensitivity 0.92 and specificity 0.87. For the MCP, with threshold decline -0.5 mm/year, AUC was 0.90 yielding sensitivity 0.85 and specificity 0.79. The Midbrain:pons ratio increased progressively in MSA-C, whereas the MCP:pons ratio was almost unchanged. AP Pons and MCP diameters were smaller in MSA-C than in MSA-P, p<0.001. We conclude from this 20-year longitudinal clinical and imaging study that AP Pons and MCP diameters are phenotypic imaging biomarkers of MSA-C. In the correct clinical context, an AP Pons and transverse MCP diameter decline of ∼0.8 mm/year is sufficient for and diagnostic of MSA-C.

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Brief Ataxia Rating Scale: A Reliable Tool to Rate Ataxia in a Short Timeframe

Cited by 11 publications

References 9 publications

Assessment of Ataxia Rating Scales and Cerebellar Functional Tests: Critique and Recommendations

Assessment of Ataxia Rating Scales and Cerebellar Functional Tests: Critique and Recommendations

Real-life ankle submovements and computer mouse use reflect patient-reported function in adult ataxias

Rates of change of pons and middle cerebellar peduncle diameters are diagnostic of multiple system atrophy of the cerebellar type

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