Subthalamic-thalamic DBS in a case with spinocerebellar ataxia type 2 and severe tremor—A unusual clinical benefit

Authors

  • Hans-Joachim Freund MD, PhD,

    Corresponding author
    1. Institute of Neuroscience and Biophysics 3, Research Center Jülich, Jülich, Germany
    2. Virtual Institute of Neuromodulation, Research Center Jülich, Jülich, Germany
    3. International Neuroscience Institute, Hannover, Germany
    • Institute of Neuroscience and Biophysics 3, Research Center Juelich, 52425 Juelich, Germany
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  • Utako B. Barnikol MD,

    1. Virtual Institute of Neuromodulation, Research Center Jülich, Jülich, Germany
    2. Department of Stereotactic and Functional Neurosurgery, University of Cologne, Cologne, Germany
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  • Dagmar Nolte PhD,

    1. Institute of Human Genetics, University of Giessen, Giessen, Germany
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  • Harald Treuer PhD,

    1. Department of Stereotactic and Functional Neurosurgery, University of Cologne, Cologne, Germany
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  • Georg Auburger MD,

    1. Section of Molecular Neurogenetics, Department of Neurology, Johann Wolfgang Goethe University, Frankfurt/Main, Germany
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  • Peter A. Tass MD, PhD,

    1. Institute of Neuroscience and Biophysics 3, Research Center Jülich, Jülich, Germany
    2. Virtual Institute of Neuromodulation, Research Center Jülich, Jülich, Germany
    3. Department of Stereotactic and Functional Neurosurgery, University of Cologne, Cologne, Germany
    4. Brain Imaging Center West, Research Center Jülich, Jülich, Germany
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  • Madjid Samii MD, PhD,

    1. International Neuroscience Institute, Hannover, Germany
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  • Volker Sturm MD, PhD

    1. Virtual Institute of Neuromodulation, Research Center Jülich, Jülich, Germany
    2. International Neuroscience Institute, Hannover, Germany
    3. Department of Stereotactic and Functional Neurosurgery, University of Cologne, Cologne, Germany
    4. Leibniz Institute for Neurobiology, Center for Learning and Memory Research, Magdeburg, Germany
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Abstract

This is a single case report of a patient with spinocerebellar ataxia type 2 (SCA2) and severe tremor. Whereas disease progression with prevailing ataxia and dysmetria was slow over the first symptomatic 6 years, 6 months prior to operation were characterized by the development of a severe, debilitating postural tremor rendering the patient unable to independently sit, stand, speak, or swallow. Deep brain stimulation (DBS) at a subthalamic–thalamic electrode position almost completely arrested her tremor. The patient regained the functional state prior to her rapid disease progression allowing a restricted range of daily activities. Her condition has remained approximately stable over the two postoperative years to date. In addition to the efficacy of DBS on cerebellar tremor, the results illustrate a remarkable improvement of the patient's general condition and independence.

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