Participants

A total of 48 patients with SCA3 and 50 sex- and age-matched healthy individuals, as the control group, participated in this study. This study was conducted in accordance with the Declaration of Helsinki and was approved by the Institutional Review Board of Taipei Veterans General Hospital. All participants provided written informed consent before participating in this study. All participants were recruited from the Department of Radiology, Taipei Veterans General Hospital. The SARA (scale for the assessment and rating of ataxia) was applied for clinical assessment of ataxia of patients with SCA3 (19). The symptoms of Parkinson's disease progress in SCA3 were measured by using Modified Hoehn and Yahr staging (20). The SARA score, which was a rating of the severity of ataxia symptoms ranging from 0 to 40, was used as a reference to indicate the progression of clinical severity in comparison with the cerebellar degeneration. A self-reported age of onset, the age at which the patients showed the first sign of any ataxic symptom, was acquired from each patient (21). The CAG repeat length of each SCA3 patient was determined by polymerase chain reaction, as described previously (22). Table 1 presents the demographic, clinical, and MR imaging data of both groups. Patients with SCA3 met the inclusion criteria if they had progressive and otherwise unexplained ataxia and tested positive for the SCA3 genotype. The disease duration in patients with SCA3 was 8.89 ± 6.432 years. Those in the control group had no central nervous system disease and did not exhibit any neurological abnormalities during the study period. An experienced neuroradiologist examined the T1- and T2-weighted images of the control group to ensure the absence of uncovered signs of another neurological disease or unexpected abnormalities.

Demographic, clinical, and MR image data of the control group and the patient group.

SARA, Scale for the Assessment and Rating of Ataxia; MMSE, Mini-Mental State Examination; H & Y staging, Hoehn and Yahr Staging Scale.