The following is a summary of “SARA captures disparate progression and responsiveness in spinocerebellar ataxias,” published in the June 2024 issue of Neurology by Petit et al.
Despite its popularity for evaluating cerebellar ataxia, the Scale for Assessment and Rating of Ataxia (SARA) has been criticized for the comprehensiveness of its scoring items.
Researchers conducted a retrospective study and analyzed data from multiple cohorts with different polyglutamine spinocerebellar ataxias (SCAs) to assess how the SARA and its components progressed, aiming to inform future clinical trials.
They analyzed data from patients with SCA1, SCA2, SCA3, and SCA6 from four cohorts (EUROSCA, RISCA, CRC-SCA, and SPATAX), totaling 850 carriers and 3431 observations. They measured the longitudinal progression of SARA and its items. They tested cohort, stage, and genetic effects, along with the respective contribution of each item to the total scale. They evaluated sensitivity to change of the scale and the impact of item removal by calculating sample sizes needed in various scenarios.
The results showed that longitudinal progression differed significantly between SCA1, SCA2, and SCA3 cohorts, with the EUROSCA cohort exhibiting the fastest progression. Patients with advanced-stage progressed slower in SCA2 and SCA6. Certain items contributed more to ataxia severity, gait, stance, hand movement, heel-shin in early stages, and finger-chase, nose-finger, and sitting in later stages. Across all populations, some items influenced the sensitivity to change of SARA; adjusting the scale structure didn’t universally improve sensitivity.
Investigators concluded that SARA item progression varied significantly across studies and SCAs, with no combination of items effectively capturing disease progression for all patient groups or SCAs.
Source: link.springer.com/article/10.1007/s00415-024-12475-1
The post SARA Explores Disparate Responsiveness and Progression in SCAs first appeared on Physician's Weekly.