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Alterations of brain metabolites in metachromatic leukodystrophy as detected by localized proton magnetic resonance spectroscopy in vivo

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Abstract

The brain morphology and chemistry of seven children with late infantile (4/7) and juvenile (3/7) forms of metachromatic leukodystrophy (MLD) were investigated by magnetic resonance imaging (MRI) and localized proton magnetic resonance spectroscopy (MRS). Patients who were examined at least 6 months after the onset of symptoms (6/7) had severe leukodystrophic changes on MRI. Proton MRS revealed a marked reduction of the neuronal markerN-acetylaspartate in white and grey matter and elevated lactate in demyelinated areas. In contrast to other leukodystrophies MLD patients showed a generalized increase of brainmyo-inositol (2- to 3-fold in white matter), indicating a specific role in the pathophysiology of demyelination in MLD.

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Authors and Affiliations

  1. Universitätskinderklinik, Schwerpunkt Neuropädiatrie, Robert-Koch-Strasse 40, D-37075, Göttingen, Germany

    Bernd Kruse, Folker Hanefeld & Hans-Jürgen Christen

  2. Max-Planck-Institut für biophysikalische Chemie, Postfach 2841, D-37085, Göttingen

    Harald Bruhn, Thomas Michaelis, Wolfgang Hänicke & Jens Frahm

Authors
  1. Bernd Kruse
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  2. Folker Hanefeld
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  3. Hans-Jürgen Christen
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  4. Harald Bruhn
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  5. Thomas Michaelis
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  6. Wolfgang Hänicke
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  7. Jens Frahm
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Kruse, B., Hanefeld, F., Christen, HJ. et al. Alterations of brain metabolites in metachromatic leukodystrophy as detected by localized proton magnetic resonance spectroscopy in vivo. J Neurol 241, 68–74 (1993). https://doi.org/10.1007/BF00869766

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  • DOI: https://doi.org/10.1007/BF00869766

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