title: Rapid onset frontal leukodystrophy with decreased diffusion coefficient and neuroaxonal spheroids.
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.
authors.affiliation: 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
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Maillart
authors.forename: Audrey
authors.initials: A
authors.lastname: Rousseau
authors.forename: Damien
authors.initials: D
authors.lastname: Galanaud
authors.forename: Françoise
authors.initials: F
authors.lastname: Gray
authors.forename: Marc
authors.initials: M
authors.lastname: Polivka
authors.forename: Pierre
authors.initials: P
authors.lastname: Labauge
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Hauw
authors.forename: Olivier
authors.initials: O
authors.lastname: Lyon-Caen
authors.forename: Olivier
authors.initials: O
authors.lastname: Gout
authors.forename: Frédéric
authors.initials: F
authors.lastname: Sedel
mesh_terms: D000328:Adult; D001927:Brain Diseases / Q000175:diagnosis / Q000473:pathology*; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D005625:Frontal Lobe / Q000473:pathology*; D006801:Humans; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D013997:Time Factors