Change in the cortical complexity of spinocerebellar ataxia type 3 appears earlier than clinical symptoms

PLoS One. 2015 Apr 21;10(4):e0118828. doi: 10.1371/journal.pone.0118828. eCollection 2015.

Abstract

Patients with spinocerebellar ataxia type 3 (SCA3) have exhibited cerebral cortical involvement and various mental deficits in previous studies. Clinically, conventional measurements, such as the Mini-Mental State Examination (MMSE) and electroencephalography (EEG), are insensitive to cerebral cortical involvement and mental deficits associated with SCA3, particularly at the early stage of the disease. We applied a three-dimensional fractal dimension (3D-FD) method, which can be used to quantify the shape complexity of cortical folding, in assessing cortical degeneration. We evaluated 48 genetically confirmed SCA3 patients by employing clinical scales and magnetic resonance imaging and using 50 healthy participants as a control group. According to the Scale for the Assessment and Rating of Ataxia (SARA), the SCA3 patients were diagnosed with cortical dysfunction in the cerebellar cortex; however, no significant difference in the cerebral cortex was observed according to the patients' MMSE ratings. Using the 3D-FD method, we determined that cortical involvement was more extensive than involvement of traditional olivopontocerebellar regions and the corticocerebellar system. Moreover, the significant correlation between decreased 3D-FD values and disease duration may indicate atrophy of the cerebellar cortex and cerebral cortex in SCA3 patients. The change of the cerebral complexity in the SCA3 patients can be detected throughout the disease duration, especially it becomes substantial at the late stage of the disease. Furthermore, we determined that atrophy of the cerebral cortex may occur earlier than changes in MMSE scores and EEG signals.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Case-Control Studies
  • Cerebellar Ataxia / pathology*
  • Cerebral Cortex / pathology*
  • Electroencephalography
  • Female
  • Humans
  • Machado-Joseph Disease / pathology*
  • Magnetic Resonance Imaging / methods*
  • Male
  • Middle Aged

Grants and funding

This work was supported by the National Science Council supported for the Center for Dynamical Biomarkers and Translational Medicine, National Central University, Taiwan (NSC 102-2911-I-008-001), Brain Research Center, National Yang-Ming University and a grant from Ministry of Education, Aim for the Top University Plan, and National Science Council (103-2221-E-010-003-MY3 and 101-2314-B-733-001-MY2 ). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.