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Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases
Published on: July 28, 2013
Rapid onset frontal leukodystrophy with decreased diffusion coefficient and neuroaxonal spheroids
Elisabeth Maillart1, Audrey Rousseau, Damien Galanaud
1Federation 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
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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