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Aims To detect specific oculomotor deficits in preclinical stage of spinocerebellar ataxia type 3 ( SCA3) and evaluate whether these abnormalities prove useful as potential biomarkers of disease progression. Methods A Chinese cohort of 56 patients with SCA3, including 12 preclinical carriers of SCA3 (pre- SCA3) and 44 manifest SCA3, and 26 healthy control individuals were recruited. We performed a detailed investigation on central oculomotor performance including fixation, gaze, smooth pursuit, prosaccade, and antisaccade using video-oculography. Results Common oculomotor features of pre- SCA3 included square-wave jerk during central fixation and gaze holding, impaired vertical smooth pursuit, slow upward saccade, and increased antisaccade error rate. In our SCA3 cohort, all oculomotor parameters were correlated with the score of the Scale for the Assessment and Rating of Ataxia, whilst some of them were correlated with disease duration. Conclusion This study showed that a series of neuropathological changes reflected by oculomotor abnormalities appeared preferentially in preclinical stage of SCA3. Accordingly, objective oculomotor preclinical signs may be useful to detect the optimum time-point for therapeutic interventions in future clinical trials of SCA3. Larger and longitudinal data are warranted to confirm our results. [ABSTRACT FROM AUTHOR]

Background: Previous studies have shown cognitive impairment in patients with spinocerebellar ataxia type 3 (SCA3). However, there is a lack of data on Chinese patients with SCA3. Method: We investigated 22 native Chinese with SCA3 and 18 controls matched for age, education as well as mental status. Cognitive assessments were carefully carried out to measure verbal fluency, memory, attention, executive function, visuospatial and visuoconstructive functions. Results: The most common impairments of cognition in native Chinese with SCA3 were disruption of phonemic verbal fluency and frontal executive dysfunction. Deficits in semantic fluency were detected in about 31.8% patients. Impaired visuospatial function and verbal memory were also found in native Chinese with SCA3. The degree of ataxia, CAG repeat length and education were found to correlate with cognitive performance. Multivariate binary logistic regression suggested that an oculomotor disorder and depression are predictors of cognitive impairment. Conclusion: Native Chinese with SCA3 had cognitive impairment of frontal executive function, temporal and parietal functions. An oculomotor disorder might be an index of cognitive dysfunction. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]