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Cerebral MRI and spectroscopy in Sjögren-Larsson syndrome: Case report

  • Diagnostic Neuroradiology
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Abstract

We report MRI and MRS of the brain in a patient with Sjögren-Larsson syndrome (SLS) in whom fatty alcohol oxidoreductase (FAO) deficiency has been verified. MRI showed periventricular lesions, high intensity on T2-weighted and low intensity on T1-weighted images at trigones of the lateral ventricles.1H-MRS of these lesions revealed high lipid and low N-acetyl aspartate peaks. We presume such lipids in periventricular lesions with high T2 signal may be pathognomonic of SLS.

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Authors and Affiliations

  1. Department of Paediatrics, Kyoto City Child Welfare Centre Shuzei-cho, Senbon Takeyamachi, Kamikyo-ku, 602, Kyoto, Japan

    Y. Miyanomae & M. Ochi

  2. Department of Paediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan

    H. Yoshioka, K. Takaya, Z. Kizaki & F. Inoue

  3. Department of Radiology, Kyoto Prefectural University of Medicine, Kyoto, Japan

    S. Furuya & S. Naruse

Authors
  1. Y. Miyanomae
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  2. M. Ochi
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  3. H. Yoshioka
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  4. K. Takaya
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  5. Z. Kizaki
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  6. F. Inoue
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  7. S. Furuya
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  8. S. Naruse
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Miyanomae, Y., Ochi, M., Yoshioka, H. et al. Cerebral MRI and spectroscopy in Sjögren-Larsson syndrome: Case report. Neuroradiology 37, 225–228 (1995). https://doi.org/10.1007/BF01578262

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  • DOI: https://doi.org/10.1007/BF01578262

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