An electrophysiological study of visual processing in spinocerebellar ataxia type 2 (SCA2)
Language English Country United States Media print
Document type Journal Article, Research Support, Non-U.S. Gov't
- MeSH
- DNA genetics MeSH
- Adult MeSH
- Electroencephalography MeSH
- Electrophysiological Phenomena MeSH
- Evoked Potentials physiology MeSH
- Middle Aged MeSH
- Humans MeSH
- Young Adult MeSH
- Neurologic Examination MeSH
- Signal Processing, Computer-Assisted MeSH
- Pattern Recognition, Visual physiology MeSH
- Aged MeSH
- Spinocerebellar Ataxias genetics physiopathology psychology MeSH
- Aging physiology MeSH
- Photic Stimulation MeSH
- Trinucleotide Repeats MeSH
- Age of Onset MeSH
- Visual Perception physiology MeSH
- Evoked Potentials, Visual physiology MeSH
- Visual Cortex physiopathology MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Young Adult MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Names of Substances
- DNA MeSH
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.
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