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

ABSTRACT:

Objective

Our 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.

Methods

We 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.

Results

2740 visits of 677 part

SUBMITTER: Jacobi H 

PROVIDER: S-EPMC10578893 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature