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Progressive cognitive dysfunction in spinocerebellar ataxia type 3

Authors

  • Sandra Roeske PhD,

    Corresponding author
    1. German Center for Neurodegenerative Diseases, Bonn, Germany
    2. Department of Neurology, University Hospital of Bonn, Bonn, Germany
    • Correspondence to: Dr. Sandra Roeske, German Center for Neurodegenerative Diseases (DZNE), Department of Clinical Research, Ernst-Robert-Curtius-Str. 12, 53117 Bonn, Germany; sandra.roeske@dzne.de

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  • Ina Filla PhD,

    1. Department of Neurology, University Hospital of Bonn, Bonn, Germany
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  • Stefan Heim PhD,

    1. Institute of Neuroscience and Medicine, Research Centre Juelich GmbH, Juelich, Germany
    2. Section Neurological Cognition Research, Department of Neurology, Medical School, Rhine-Westphalia Technical University, Aachen University, Aachen, Germany
    3. Section Structural Functional Brain Mapping, Department of Psychiatry, Psychotherapy and Psychosomatics, Medical School, Rhine-Westphalia Technical University, Aachen University, Aachen, Germany
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  • Katrin Amunts MD,

    1. Institute of Neuroscience and Medicine, Research Centre Juelich GmbH, Juelich, Germany
    2. C. & O. Vogt Institute for Brain Research, Heinrich Heine University Düsseldorf, Düsseldorf, Germany
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  • Christoph Helmstaedter PhD,

    1. Department of Epileptology-Neuropsychology, University Hospital of Bonn, Bonn, Germany
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  • Ullrich Wüllner MD,

    1. Department of Neurology, University Hospital of Bonn, Bonn, Germany
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  • Michael Wagner PhD,

    1. Department of Psychiatry and Psychotherapy-Neuropsychology, University Hospital of Bonn, Bonn, Germany
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  • Thomas Klockgether MD,

    1. German Center for Neurodegenerative Diseases, Bonn, Germany
    2. Department of Neurology, University Hospital of Bonn, Bonn, Germany
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  • Martina Minnerop MD

    1. Department of Neurology, University Hospital of Bonn, Bonn, Germany
    2. Institute of Neuroscience and Medicine, Research Centre Juelich GmbH, Juelich, Germany
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  • Funding agencies: Data acquisition for this study at baseline was part of a project supported by the Deutsche Forschungs-Gemeinschaft (DFG, WU184/3-1) and BMBF (BICW 01 GO 0515). Data aquisition at follow-up was funded in part by the Maria von Linden-Support Programme of the University of Bonn, Germany (to M.M.).

  • Relevant conflicts of interest/financial disclosures: Nothing to report.

  • Full financial disclosures and author roles may be found in the online version of this article.

ABSTRACT

Background

Although it is well established that there is cognitive dysfunction in spinocerebellar ataxia type 3 (SCA3), it is unknown whether cognition deteriorates with disease progression. We therefore prospectively studied cognitive function in patients with SCA3.

Methods

Eleven patients with SCA3 were assessed using an extensive neuropsychological test battery and retested after 3.5 ± 0.4 years.

Results

In addition to ataxia and motor control, verbal learning and verbal and figural memory deteriorated significantly during the follow-up period. An increase in depressive symptoms was not observed.

Conclusions

The observation that memory and learning abilities deteriorated with disease progression suggests that cognitive dysfunction is an integral part of SCA3. Because the applied tests for memory function did not require motor responses, cognitive decline cannot be attributed to progressive cerebellar ataxia. The deterioration of verbal and figural memory can be explained either by extracerebellar pathology or by disruption of cerebellar-cerebral circuitries. © 2013 International Parkinson and Movement Disorder Society

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