Vascular Risk Factors and Clinical Progression in Spinocerebellar Ataxias

  • Raymond Y. Lo
  • Karla P. Figueroa
  • Stefan M. Pulst
  • Chi-Ying Lin
  • Susan Perlman
  • George Wilmot
  • Christopher M. Gomez
  • Jeremy Schmahmann
  • Henry Paulson
  • Vikram G. Shakkottai
  • Sarah H. Ying
  • Theresa Zesiewicz
  • Khalaf Bushara
  • Michael Geschwind
  • Guangbin Xia
  • S S. H. Subramony
  • Tetsuo Ashizawa
  • Sheng-Han Kuo

Abstract

Background: The contributions of vascular risk factors to spinocerebellar ataxia (SCA) are not known.

Methods: We studied 319 participants with SCA 1, 2, 3, and 6 and repeatedly measured clinical severity using the Scale for Assessment and Rating of Ataxia (SARA) for 2 years. Vascular risk factors were summarized by CHA2DS2-VASc scores as the vascular risk factor index. We employed regression models to study the effects of vascular risk factors on ataxia onset and progression after adjusting for age, sex, and pathological CAG repeats. Our secondary analyses took hyperlipidemia into account.

Results: Nearly 60% of SCA participants were at low vascular risks with CHA2DS2-VASc = 0, and 31% scored 2 or greater. Higher CHA2DS2-VASc scores were not associated with either earlier onset or faster progression of ataxia. These findings were not altered after accounting for hyperlipidemia.

Discussion: Vascular risks are not common in SCAs and are not associated with earlier onset or faster ataxia progression.