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Cranial magnetic resonance imaging in genetically proven myotonic dystrophy type 1 and 2

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Abstract.

Cranial magnetic resonance imaging (MRI) in 19 German patients with genetically proven myotonic dystrophy Type 1 (DM1, n = 10) or Type 2 (DM2, n = 9) showed pathological findings consisting of white matter lesions (WML) and/or brain atrophy in 9/10 DM1 and 8/9 DM2 patients. Anterior temporal WML (ATWML) were exclusively seen in DM1 patients. Our findings indicate a high frequency of central nervous system (CNS) involvement in both disorders. However, temporopolar pathology, previously associated with intellectual dysfunction, seems to be restricted to DM1.

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Authors and Affiliations

  1. Dept. of Neurology, University of Bonn, Sigmund-Freud-Straße 25, 53105, Bonn, Germany

    Cornelia Kornblum, Christoph Grothe, Niki Amanatidis, Thomas Klockgether & Rolf Schröder MD

  2. Dept. of Neuroradiology, Medizin Centrum Bonn, Bonn, Germany

    Jürgen Reul

  3. Dept. of Human Genetics, University of Würzburg, Würzburg, Germany

    Wolfram Kress

Authors
  1. Cornelia Kornblum
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  2. Jürgen Reul
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  3. Wolfram Kress
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  4. Christoph Grothe
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  5. Niki Amanatidis
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  6. Thomas Klockgether
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  7. Rolf Schröder MD
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Corresponding author

Correspondence to Rolf Schröder MD.

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Kornblum, C., Reul, J., Kress, W. et al. Cranial magnetic resonance imaging in genetically proven myotonic dystrophy type 1 and 2. J Neurol 251, 710–714 (2004). https://doi.org/10.1007/s00415-004-0408-1

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  • DOI: https://doi.org/10.1007/s00415-004-0408-1

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