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Rapid onset frontal leukodystrophy with decreased diffusion coefficient and neuroaxonal spheroids

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Abstract

Adult leukodystrophies with neuroaxonal spheroids (LNS) constitute a heterogeneous group of genetic diseases. Herein, we report on two unrelated patients with LNS characterized by rapid onset, predominant involvement of the frontal white matter, and areas of decreased apparent diffusion coefficient on diffusion-weighted imaging. We found similar cases in the literature and propose that they represent a distinct entity within the group of LNS. Further studies will be required to identify its molecular basis.

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Authors and Affiliations

  1. Federation of Nervous System Diseases, Hôpital de la Salpêtrière, Assistance Publique-Hôpitaux de Paris, Université Pierre et Marie Curie (Paris VI), 47 Boulevard de l’Hôpital, 75651, Paris cedex 13, France

    Elisabeth Maillart, Olivier Lyon-Caen & Frédéric Sedel

  2. Neuropathology Department, Hôpital de la Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France

    Audrey Rousseau & Jean-Jacques Hauw

  3. Neuroimagery Department, Hôpital de la Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France

    Damien Galanaud

  4. Department of Pathology, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, Université Paris VII, Paris, France

    Françoise Gray & Marc Polivka

  5. Neurology Department, GHU, Nimes, France

    Pierre Labauge

  6. Neurology Department, Fondation Ophtalmologique de Rothschild, Paris, France

    Olivier Gout

Authors
  1. Elisabeth Maillart
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  2. Audrey Rousseau
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  3. Damien Galanaud
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  4. Françoise Gray
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  5. Marc Polivka
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  6. Pierre Labauge
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  7. Jean-Jacques Hauw
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  8. Olivier Lyon-Caen
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  9. Olivier Gout
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  10. Frédéric Sedel
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Correspondence to Elisabeth Maillart.

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Maillart, E., Rousseau, A., Galanaud, D. et al. Rapid onset frontal leukodystrophy with decreased diffusion coefficient and neuroaxonal spheroids. J Neurol 256, 1649–1654 (2009). https://doi.org/10.1007/s00415-009-5172-9

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  • DOI: https://doi.org/10.1007/s00415-009-5172-9

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