Scale for the assessment and rating of ataxia: Age‐dependent performance of healthy adults

Grobe‐Einsler, Marcus; Schmidt, Alina; Schaprian, Tamara; Vogt, Ina R.; Klockgether, Thomas

doi:10.1111/ene.15596

Cited by 4 publications

(4 citation statements)

References 7 publications

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“…However, they allowed to estimate at which time SCA mutation carriers cross the threshold of low COA scores, such as SARA = 1, which are still in the normal range. The apparently earlier deterioration of SCA6 mutation carriers, who were 20 to 25 years older than SCA1, SCA2 and SCA3 mutation carriers, is most likely an artefact due to age‐related higher SARA scores 28 …”

Section: Discussionmentioning

confidence: 99%

“…The apparently earlier deterioration of SCA6 mutation carriers, who were 20 to 25 years older than SCA1, SCA2 and SCA3 mutation carriers, is most likely an artefact due to age-related higher SARA scores. 28 The rationale for focusing on this time period is that initiation of therapies at this time will precede irreversible brain damage occurring in the later disease course. Accordingly, a recently established trial-ready cohort for SCA1 and SCA3 specifically enrolled pre-ataxic mutation carriers and mildly affected patients.…”

Section: Discussionmentioning

confidence: 99%

“…The apparently earlier deterioration of SCA6 mutation carriers, who were 20 to 25 years older than SCA1, SCA2 and SCA3 mutation carriers, is most likely an artefact due to age‐related higher SARA scores. 28 …”

Section: Discussionmentioning

confidence: 99%

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Disease progression of spinocerebellar ataxia types 1, 2, 3 and 6 before and after ataxia onset

Jacobi,

Schaprian,

Schmitz‐Hübsch

et al. 2023

Ann Clin Transl Neurol

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ObjectiveOur aim was to study the evolution of ataxia and neurological symptoms before and after ataxia onset in the most common spinocerebellar ataxias (SCAs), SCA1, SCA2, SCA3 and SCA6. We therefore jointly analysed the data of the EUROSCA and RISCA studies, which recruited ataxic and non‐ataxic mutation carriers.MethodsWe used mixed effect models to analyse the evolution of Scale for the Rating and Assessment of Ataxia (SARA) scores, SCA Functional Index (SCAFI) and Inventory of Non‐Ataxia Signs (INAS) counts. We applied multivariable modelling to identify factors associated with SARA progression. In the time interval 5 years prior to and after ataxia onset, we calculated sensitivity to change ratios (SCS) of SARA, SCAFI and INAS.Results2740 visits of 677 participants were analysed. All measures showed non‐linear progression that was best fitted by linear mixed models with linear, quadratic and cubic time effects. R2 values indicating quality of the fit ranged from 0.70 to 0.97. CAG repeat was associated with faster progression in SCA1, SCA2 and SCA3, but not SCA6. 5 years prior to and after ataxia onset, SARA had the highest SCS of all measures with a mean of 1.21 (95% CI: 1.20, 1.21) in SCA1, 0.94 (0.93, 0.94) in SCA2 and 1.23 (1.22, 1.23) in SCA3.InterpretationOur data have important implications for the understanding of disease progression in SCA1, SCA2, SCA3 and SCA6 across the lifespan. Furthermore, our study provides information for the design of interventional trials, especially in pre‐ataxic mutation carriers close to ataxia onset and patients in early disease stages.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Disease progression of spinocerebellar ataxia types 1, 2, 3 and 6 before and after ataxia onset

Jacobi,

Schaprian,

Schmitz‐Hübsch

et al. 2023

Ann Clin Transl Neurol

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“…Score ranges from 0 to 40. SARA has been validated for assessing early onset ataxias 18‐20 Gait Scale (GS) where a higher score reflects better function, which ranges from 0 to 10 21 …”

Section: Methodsmentioning

confidence: 99%

Long‐Term Nicotinamide Riboside Use Improves Coordination and Eye Movements in Ataxia Telangiectasia

Presterud,

Deng,

Wennerström

et al. 2023

Movement Disorders

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BackgroundSupplementation of nicotinamide riboside (NR) ameliorates neuropathology in animal models of ataxia telangiectasia (A‐T). In humans, short‐term NR supplementation showed benefits in neurological outcome.ObjectivesThe study aimed to investigate the safety and benefits of long‐term NR supplementation in individuals with A‐T.MethodsA single‐arm, open‐label clinical trial was performed in individuals with A‐T, receiving NR over a period of 2 years. Biomarkers and clinical examinations were used to assess safety parameters. Standardized and validated neuromotor tests were used to monitor changes in neurological symptoms. Using generalized mixed models, test results were compared to expected disease progression based on historical data.ResultsNAD+ concentrations increased rapidly in peripheral blood and stabilized at a higher level than baseline. NR supplementation was well tolerated for most participants. The total scores in the neuromotor test panels, as evaluated at the 18‐month time point, improved for all but one participant, primarily driven by improvements in coordination subscores and eye movements. A comparison with historical data revealed that the progression of certain neuromotor symptoms was slower than anticipated.ConclusionsLong‐term use of NR appears to be safe and well tolerated, and it improves motor coordination and eye movements in patients with A‐T of all ages. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

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Physical Activity and Fitness Levels of Individuals With Ataxia: A Cross-Sectional Study

Barbuto,

Lee,

Stein

et al. 2023

Archives of Physical Medicine and Rehabilitation

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Scale for the assessment and rating of ataxia: Age‐dependent performance of healthy adults

Cited by 4 publications

References 7 publications

Disease progression of spinocerebellar ataxia types 1, 2, 3 and 6 before and after ataxia onset

Disease progression of spinocerebellar ataxia types 1, 2, 3 and 6 before and after ataxia onset

Long‐Term Nicotinamide Riboside Use Improves Coordination and Eye Movements in Ataxia Telangiectasia

Physical Activity and Fitness Levels of Individuals With Ataxia: A Cross-Sectional Study

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