• Journal of neurology · Dec 1993

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

    • B Kruse, F Hanefeld, H J Christen, H Bruhn, T Michaelis, W Hänicke, and J Frahm.
    • Universitätskinderklinik, Schwerpunkt Neuropädiatrie, Göttingen, Germany.
    • J. Neurol. 1993 Dec 1; 241 (2): 68-74.

    AbstractThe 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 marker N-acetylaspartate in white and grey matter and elevated lactate in demyelinated areas. In contrast to other leukodystrophies MLD patients showed a generalized increase of brain myo-inositol (2- to 3-fold in white matter), indicating a specific role in the pathophysiology of demyelination in MLD.

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