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Rapid onset frontal leukodystrophy with decreased diffusion coefficient and neuroaxonal spheroids. 2009
Elisabeth Maillart, and
Audrey Rousseau, and
Damien Galanaud, and
Françoise Gray, and
Marc Polivka, and
Pierre Labauge, and
Jean-Jacques Hauw, and
Olivier Lyon-Caen, and
Olivier Gout, and
Frédéric Sedel
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. elisabethmaillart@yahoo.fr
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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Rinsho shinkeigaku = Clinical neurology,
