[Fulminant orthochromatic leukodystrophy in an adult].
A 27-year-old man from Marocco developed a progressive dementia leading within a year to a mutic akinetic state. The course of the disease was also marked by epileptic seizures. MRI revealed diffuse white matter involvement. A frontal white matter brain biopsy was consistent with the diagnosis of orthochromatic leucodystrophy, i.e: presence of sudanophilic lipids and pigmented cells associated with myelin loss. Adult forms of orthochromatic leucodystrophy are very rare. Our case was characterized by a fulminant course.
Dousset
V
V
Service de Neurologie, Hôpital Pellegrin, Bordeaux.
Tison
F
F
Vital
A
A
Henry
P
P
D000328:Adult; D001921:Brain / Q000473:pathology*; D001927:Brain Diseases / Q000175:diagnosis* / Q000473:pathology; D003704:Dementia; D003711:Demyelinating Diseases / Q000175:diagnosis* / Q000473:pathology; D004827:Epilepsy; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests
Leucodystrophie orthochromatique d'évolution rapide chez l'adulte.