Item performance of the scale for the assessment and rating of ataxia in rare and ultra-rare genetic ataxias.

ABSTRACT: The Scale for the Assessment and Rating of Ataxia (SARA) is widely used for assessing the severity and progression of genetic cerebellar ataxias. SARA is now considered a primary end point in several ataxia treatment trials, but its underlying composite item measurement model has not yet been tested. This work aimed to evaluate the composite properties of SARA and its items using item response theory (IRT) and to demonstrate its applicability across even ultra-rare genetic ataxias. Leveraging SARA subscores data from 1932 visits from 990 patients of the Autosomal Recessive Cerebellar Ataxias (ARCA) registry, we assessed the performance of SARA using IRT methodology. The item characteristics were evaluated over the ataxia severity range of the entire ataxia population as well as the assessm

SUBMITTER: Hamdan A 

PROVIDER: S-EPMC11330187 | biostudies-literature | 2024 Aug

REPOSITORIES: biostudies-literature