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Extensive white-matter changes in case of adult polyglucosan body disease

  • DIAGNOSTIC NEURORADIOLOGY
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Abstract

Extensive white matter signal changes were observed on T2-weighted images of a 49-year-old man. He presented with a slowly progressive gait disorder, and finally developed severe dementia. Extensive metabolic and infectious investigations failed to disclose the underlying cause during life. Autopsy revealed adult polyglucosan body disease. We discuss MRI findings likely to permit this diagnosis if combined with clinical findings and nerve or skin biopsy.

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Authors and Affiliations

  1. Department of Neurology, Inselspital, Murtenstrasse, 3010 Berne, Switzerland e-mail: matthias.sturzenegger@insel.ch Tel.: + 41-31-6 32 30 66 Fax: + 41-31-6 32 96 79, , , , , , CH

    M. Berkhoff & M. Sturzenegger

  2. Department of Neuroradiology, University of Berne, 3010 Berne, Switzerland, , , , , , CH

    G. Schroth

  3. Department of Neuropathology, University of Berne, 3010 Berne, Switzerland, , , , , , CH

    J. Weis

Authors
  1. M. Berkhoff
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  2. J. Weis
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  3. G. Schroth
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  4. M. Sturzenegger
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Received: 14 July 2000 Accepted: 5 September 2000

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Berkhoff, M., Weis, J., Schroth, G. et al. Extensive white-matter changes in case of adult polyglucosan body disease. Neuroradiology 43, 234–236 (2001). https://doi.org/10.1007/s002340000425

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  • DOI: https://doi.org/10.1007/s002340000425

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