[Cavitary orthochromatic leukodystrophy with oligodendroglial changes. A sporadic adult case]

A 39 year-old man with mild stable mental retardation, without family history, developed progressively a gait disturbance and intellectual deterioration. CT scan showed a low density of the periventricular hemispheric white matter which increased on subsequent examinations. Eight months before death he presented with several Grand Mal seizures. He died 29 months after the onset of the clinical disorders. Neuropathological studies included light and electron microscopy of a cerebral biopsy and a post-mortem examination of the brain. It showed a sudanophilic leukodystrophy with unusual features: cavitation of the white matter, oligodendrocyte proliferation and lamellar "fingerprint" dense cytoplasmic inclusions in the oligodendrocytes. Only 3 similar cases have been previously reported.