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Localised proton magnetic resonance spectroscopy in X-linked adrenoleukodystrophy

  • Paediatric Neuroradiology
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Abstract

We have performed localised proton magnetic resonance spectroscopy (MRS) of the brain on four patients with X-linked adrenoleukodystrophy (X-ALD). The spectrum is characterised at the beginning of the disease by a decrease in N-acetylaspartate and phosphocreatine-creatine content. Choline is strongly increased, and lactate can be detected in some cases. A proton signal from the CH2 groups borne by free intracellular very long chain fatty acids can also be observed. Later in the disease, the levels of all metabolites, in particular NAA, decrease significantly. The progression of neurometabolism documented by MRS correlates well with MRI and clinical progression on follow-up study. In one case, the metabolic profile recorded by proton MRS was abnormal before any change occurred on MRI. Proton MRS of the brain might be the method of choice for monitoring patients with X-ALD, to screen presumed cases and to study the effects of treatment.

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Authors and Affiliations

  1. URA CNRS 1186, Faculté de Médicine de Marseille, Centre de Résonance Magnétique Biologique et Médicale (CRMBM), 27 Bd J. Moulin, F-13005, Marseille, France

    S. Confort-Gouny, J. Vion-Dury & P. J. Cozzone

  2. Service de Neuropédiatrie, Hôpital d'Enfants, Centre Hospitalo Universitaire de la Timone, 13385, Cedex 5 Marseille, France

    B. Chabrol

  3. Service de Neurologie, Hôpital Ste Marguerite, 13009, Marseille, France

    F. Nicoli

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  1. S. Confort-Gouny
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  2. J. Vion-Dury
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  3. B. Chabrol
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  4. F. Nicoli
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  5. P. J. Cozzone
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Confort-Gouny, S., Vion-Dury, J., Chabrol, B. et al. Localised proton magnetic resonance spectroscopy in X-linked adrenoleukodystrophy. Neuroradiology 37, 568–575 (1995). https://doi.org/10.1007/BF00593724

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  • DOI: https://doi.org/10.1007/BF00593724

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