Affordable Access

deepdyve-link
Publisher Website

Static and Dynamic Ocular Motor Abnormalities as Potential Biomarkers in Spinocerebellar Ataxia Type 3.

Authors
  • Lemos, João1, 2
  • Novo, Ana3
  • Duque, Cristina3
  • Cunha, Inês3
  • Ribeiro, Joana3
  • Castelhano, João4
  • Januário, Cristina3, 5
  • 1 Neurology Department, Coimbra University Hospital Centre, Praceta Mota Pinto, 3000-135, Coimbra, Portugal. [email protected] , (Portugal)
  • 2 Coimbra Institute for Clinical and Biomedical Research, Faculty of Medicine, Coimbra University, Coimbra, Portugal. [email protected] , (Portugal)
  • 3 Neurology Department, Coimbra University Hospital Centre, Praceta Mota Pinto, 3000-135, Coimbra, Portugal. , (Portugal)
  • 4 Coimbra Institute for Clinical and Biomedical Research, Faculty of Medicine, Coimbra University, Coimbra, Portugal. , (Portugal)
  • 5 Faculty of Medicine, Coimbra University, Coimbra, Portugal. , (Portugal)
Type
Published Article
Journal
Cerebellum (London, England)
Publication Date
Jun 01, 2021
Volume
20
Issue
3
Pages
402–409
Identifiers
DOI: 10.1007/s12311-020-01217-4
PMID: 33215370
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

While dynamic ocular motor abnormalities (e.g., gaze-evoked nystagmus (GEN), low optokinetic nystagmus (OKN), pursuit and vestibulo-ocular reflex (VOR) gains, and dysmetric saccades) have been shown to be potential biomarkers in spinocerebellar ataxia type 3 (SCA3), the value of static abnormalities (e.g., convergent [esodeviation] and divergent strabismus [exodeviation]) is unknown. Moreover, studies on dynamic abnormalities in SCA3 usually do not take into account the existence of potential abduction-adduction asymmetries in patients with degenerative ataxia. Thirty-eight patients with genetically confirmed SCA3 (24 females; mean age ± SD, 49.8± 12.2 years) and 22 healthy controls (12 females, p = 0.589; mean age ± SD, 50.7± 12.5 years, p = 0.651) underwent clinical and video-oculographic assessment. A p value < 0.002 (between- and within-group analyses) and < 0.001 (correlation analysis) was considered significant. Patients showed larger esodeviation at distance (p < 0.001), became more esodeviated in lateral gaze (p < 0.001), and their near exodeviation correlated with scale for the assessment and rating of ataxia (SARA) score (p = 0.004). Pursuit, OKN, and VOR gains were lower in patients, both for their adducting and abducting components (p < 0.001). Saccades showed higher velocities (p < 0.001), abducting saccades showed lower amplitude (p < 0.001), and adducting saccades tended to show greater vertical bias (p = 0.018) in patients. Abducting saccades showed relatively lower velocity (p < 0.001) and lower amplitude (p = 0.015) than abducting saccades within patients. All dynamic ocular motor abnormalities except saccades correlated with SARA score, CAG repeat number, and/or disease duration (p < 0.001). Static and dynamic ocular motor abnormalities are potential biomarkers in SCA3. SCA3 studies using saccades should take into account the existence of potential abduction-adduction asymmetries.

Report this publication

Statistics

Seen <100 times