FULMINANT ADULT ORTHOCHROMATIC LEUKODYSTROPHY

A 27 year-old man from Marocco developed a progressive dementia leadin g within a year to a mutic akinetic state. The course of the disease w as also marked by epileptic seizures. MRI revealed diffuse white matte r involvement. A frontal white matter brain biopsy was consistent with the diagnosis of orthochromatic leucodystrophy, i.e: presence of suda nophilic lipids and pigmented cells associated with myelin loss. Adult forms of orthochromatic leucodystrophy are very rare. Our case was ch aracterized by a fulminant course.