Abstract

Introduction: Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant, cerebellar degeneration predominant disease caused by excessive CAG repeats. We examined event-related dysynchronization/synchronization (ERD/ERS) in patients with SCA3.Methods: We assessed ERD/ERS of self-paced voluntary hand movements in 15 patients with genetically proven SCA3 in comparison with healthy controls.Results: In ERS, a significant interaction effect between group, frequency, and period (F = 1.591; p = 0.005; ρI = 0.86) was observed. The post-hoc two-tailed independent t-test showed significant differences in high beta and low beta ERS. By contrast, in ERD, no apparent differences were observed in the pattern of patients with SCA3 in comparison with healthy controls (F = 1.01; p = 0.442).Conclusion: The study revealed a decreased ERS in patients with SCA3, especially at the frequency of 20–30 Hz. This study elucidates the significant role of cerebellum in motor control.

Highlights

  • Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant, cerebellar degeneration predominant disease caused by excessive CAG repeats

  • In event-related desynchronization (ERD), no apparent differences were observed in the pattern of patients with SCA3 in comparison with healthy controls (F = 1.01; p = 0.442)

  • This study elucidates the significant role of cerebellum in motor control

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Summary

Introduction

Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant, cerebellar degeneration predominant disease caused by excessive CAG repeats. A previous study on movement-related cortical potentials (MRCP) in patients with SCA3 revealed impaired cortical activation associated with affects the initiation and termination of voluntary movements [3]. It suggested an impairment of the postsynaptic potentials on the pyramidal cell apical dendrites. The analysis of electroencephalogram (EEG) oscillation recorded during a motor task (self-paced movement) provided different information about the changes in ERD/ERS in Spinocerebellar Ataxia Type 3 cortical activity related to movement [4, 5], especially the neurons that involved in cortico-thalamic oscillations or other remote connectivities. To the best of our knowledge, this is the first experiment aiming at understanding the ERD/ERS pattern changes in patients with SCA3

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