Rapid onset frontal leukodystrophy with decreased diffusion coefficient and neuroaxonal spheroids.
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.
Maillart
Elisabeth
E
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
Rousseau
Audrey
A
Galanaud
Damien
D
Gray
Françoise
F
Polivka
Marc
M
Labauge
Pierre
P
Hauw
Jean-Jacques
JJ
Lyon-Caen
Olivier
O
Gout
Olivier
O
Sedel
Frédéric
F
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