Differential Temporal Dynamics of Axial and Appendicular Ataxia in SCA3.

ABSTRACT:

Background

Disease severity in spinocerebellar ataxia type 3 (SCA3) is commonly defined by the Scale for the Assessment and Rating of Ataxia (SARA) sum score, but little is known about the contributions and progression patterns of individual items.

Objectives

To investigate the temporal dynamics of SARA item scores in SCA3 patients and evaluate if clinical and demographic factors are differentially associated with evolution of axial and appendicular ataxia.

Methods

In a prospective, multinational cohort study involving 11 European and 2 US sites, SARA scores were determined longitudinally in 223 SCA3 patients with a follow-up assessment after 1 year.

Results

An increase in SARA score from 10 to 20 points was mainly driven by axial and speech items, with a marke

SUBMITTER: Maas RPPWM 

PROVIDER: S-EPMC9540189 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature