The Cerebellum

, Volume 10, Issue 1, pp 32–42 | Cite as

An Electrophysiological Study of Visual Processing in Spinocerebellar Ataxia Type 2 (SCA2)

  • Jan Kremlacek
  • Martin Valis
  • Jiri Masopust
  • Ales Urban
  • Alena Zumrova
  • Radomir Talab
  • Miroslav Kuba
  • Zuzana Kubova
  • Jana Langrova


Reports of visual functional impairment in spinocerebellar ataxia type 2 (SCA2) have been studied previously using pattern reversal visually evoked potentials (VEPs) with contradictory results. To provide additional evidence to this area, visual functions were studied using VEPs and event-related potentials (ERPs) in a group of ten patients with genetically verified SCA2. The electrophysiological examination included pattern reversal and motion-onset VEPs as well as visually driven oddball ERPs with an evaluation of a target and a pre-attentive response. In six patients, we found abnormal visual/cognitive processing that differed from normal values in latency, but not in the amplitude of the dominant VEP/ERP peaks. Among the VEPs/ERPs used, the motion-onset VEPs exhibited the highest sensitivity and showed a strong Spearman correlation to SCA2 duration (from r = 0.82 to r = 0.90, p < 0.001) and clinical state assessed by Brief Ataxia Rating Scale (from r = 0.71 (p = 0.022) to r = 0.80 (p < 0.001)). None of the VEP/ERP latencies showed a correlation to the triplet repeats of the SCA2 gene. In three patients, we did not find any visual/cognitive pathology, and one subject showed only a single subtle prolongation of the VEP peak. The observed visual/cognitive deficit was related to the subjects’ clinical state and the illness duration, but no relationship to the genetic marker of SCA2 was found. From the VEP/ERP types used, the motion-onset VEPs seems to be the most promising candidate for clinical state monitoring rather than a tool for early diagnostic use.


Spinocerebellar ataxia type 2 Visual evoked potentials Event-related potentials Motion processing 



This study is supported by Grant Agency of the Czech Republic 309/09/0869.

Conflict of Interest

There is no conflict of interest and all funding sources supporting the work and all institutional or corporate affiliations of ours are acknowledged.


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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Jan Kremlacek
    • 1
    • 2
  • Martin Valis
    • 2
  • Jiri Masopust
    • 3
  • Ales Urban
    • 3
  • Alena Zumrova
    • 4
  • Radomir Talab
    • 2
  • Miroslav Kuba
    • 1
  • Zuzana Kubova
    • 1
  • Jana Langrova
    • 1
  1. 1.Department of Pathophysiology, Faculty of MedicineCharles University in PragueHradec KraloveCzech Republic
  2. 2.Department of Neurology, Faculty of MedicineCharles University in Prague, University Hospital Hradec KraloveHradec KraloveCzech Republic
  3. 3.Department of Psychiatry, Faculty of MedicineCharles University in Prague, University Hospital Hradec KraloveHradec KraloveCzech Republic
  4. 4.Neurogenetic Centre, 2nd Faculty of Medicine of Charles University and Faculty Hospital MotolPragueCzech Republic

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