Relevant conflicts of interest/financial disclosures: Nothing to report.
Ocular motor characteristics of different subtypes of spinocerebellar ataxia: Distinguishing features
Article first published online: 22 APR 2013
Copyright © 2013 Movement Disorder Society
Volume 28, Issue 9, pages 1271–1277, August 2013
How to Cite
Kim, J. S., Kim, J. S., Youn, J., Seo, D.-W., Jeong, Y., Kang, J.-H., Park, J. H. and Cho, J. W. (2013), Ocular motor characteristics of different subtypes of spinocerebellar ataxia: Distinguishing features. Mov. Disord., 28: 1271–1277. doi: 10.1002/mds.25464
Full financial disclosures and author roles may be found in the online version of this article.
- Issue published online: 3 SEP 2013
- Article first published online: 22 APR 2013
- Manuscript Accepted: 15 FEB 2013
- Manuscript Revised: 24 JAN 2013
- Manuscript Received: 18 SEP 2012
- spinocerebellar ataxia;
- eye movements;
Because of frequent involvement of the cerebellum and brainstem, ocular motor abnormalities are key features of spinocerebellar ataxias and may aid in differential diagnosis. Our objective for this study was to distinguish the subtypes by ophthalmologic features after head-shaking and positional maneuvers, which are not yet recognized as differential diagnostic tools in most common forms of spinocerebellar ataxias. Of the 302 patients with a diagnosis of cerebellar ataxia in 3 Korean University Hospitals from June 2011 to June 2012, 48 patients with spinocerebellar ataxia types 1, 2, 3, 6, 7, or 8 or with undetermined spinocerebellar ataxias were enrolled. All patients underwent a video-oculographic recording of fixation abnormalities, gaze-evoked nystagmus, positional and head-shaking nystagmus, and dysmetric saccades. Logistic regression analysis controlling for disease duration revealed that spontaneous and positional downbeat nystagmus and perverted head-shaking nystagmus were strong predictors for spinocerebellar ataxia 6, whereas saccadic intrusions and oscillations were identified as positive indicators of spinocerebellar ataxia 3. In contrast, the presence of gaze-evoked nystagmus and dysmetric saccades was a negative predictor of spinocerebellar ataxia 2. Positional maneuvers and horizontal head shaking occasionally induced or augmented saccadic intrusions/oscillations in patients with spinocerebellar ataxia types 1, 2, and 3 and undetermined spinocerebellar ataxia. The results indicated that perverted head-shaking nystagmus may be the most sensitive parameter for SCA6, whereas saccadic intrusions/oscillations are the most sensitive for spinocerebellar ataxia 3. In contrast, a paucity of gaze-evoked nystagmus and dysmetric saccades is more indicative of spinocerebellar ataxia 2. Head-shaking and positional maneuvers aid in defining ocular motor characteristics in spinocerebellar ataxias. © 2013 Movement Disorder Society